MONDO:0001071 |
MONDO:0001071
Disease Name: intellectual disability
Orphanet id: Orphanet:319658
All associated enzymes in DAR:
CYP2U1
DHX16
CBS
POLG
UFSP2
P4HTM
PDIA3
DHX30
TET3
ALG3
NAGLU
CHD3
SMPD1
DHX34
GCH1
CARNMT1
CYP27A1
ABCD1
CHD2
DDX54
INO80
PTPN4
DHX37
SMPD1 |
P17405 |
3.1.4.12
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1605797
(
R-HSA-1660662
)
R-HSA-1605797
Reaction Name: Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome)
See in Reactome
|
MONDO:0002049 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0007488 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0007847 |
ASAH1 |
Q13510 |
3.5.1.23
3.5.1.-
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1606602
(
R-HSA-1660662
)
R-HSA-1606602
Reaction Name: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
See in Reactome
R-HSA-6798745
(
R-HSA-6798695
)
R-HSA-6798745
Reaction Name: Exocytosis of tertiary granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0008045 |
ASAH1 |
Q13510 |
3.5.1.23
3.5.1.-
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1606602
(
R-HSA-1660662
)
R-HSA-1606602
Reaction Name: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
See in Reactome
R-HSA-6798745
(
R-HSA-6798695
)
R-HSA-6798745
Reaction Name: Exocytosis of tertiary granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0008199 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0008779 |
MONDO:0008779
Disease Name: arthrogryposis
All associated enzymes in DAR:
GBE1
EARS2
ASAH1
ATP2B3
ASAH1 |
Q13510 |
3.5.1.23
3.5.1.-
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1606602
(
R-HSA-1660662
)
R-HSA-1606602
Reaction Name: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
See in Reactome
R-HSA-6798745
(
R-HSA-6798695
)
R-HSA-6798745
Reaction Name: Exocytosis of tertiary granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0009218 |
ASAH1 |
Q13510 |
3.5.1.23
3.5.1.-
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1606602
(
R-HSA-1660662
)
R-HSA-1606602
Reaction Name: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
See in Reactome
R-HSA-6798745
(
R-HSA-6798695
)
R-HSA-6798745
Reaction Name: Exocytosis of tertiary granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0009260 |
GLB1 |
P16278 |
3.2.1.23
|
R-HSA-2022857
R-HSA-2024096
R-HSA-1660662
R-HSA-6798695
R-HSA-4085001
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-1638091
R-HSA-1638091
Reactome Name: Heparan sulfate/heparin (HS-GAG) metabolism
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605624
(
R-HSA-1660662
)
R-HSA-1605624
Reaction Name: Beta-galactosidase hydrolyses GM1 to GM2
See in Reactome
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-1606312
(
R-HSA-1660662
)
R-HSA-1606312
Reaction Name: Beta-galactosidase can also hydrolyse globosides to form cerebrosides
See in Reactome
R-HSA-1630306
(
R-HSA-2022857
)
R-HSA-1630306
Reaction Name: GLB1 hydrolyses a glycosaminoglycan
See in Reactome
R-HSA-2090079
(
R-HSA-2024096
)
R-HSA-2090079
Reaction Name: GLB1 hydrolyses linker chain(2)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
R-HSA-9638120
(
R-HSA-4085001
)
R-HSA-9638120
Reaction Name: NEU4 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
|
MONDO:0009261 |
GLB1 |
P16278 |
3.2.1.23
|
R-HSA-2022857
R-HSA-2024096
R-HSA-1660662
R-HSA-6798695
R-HSA-4085001
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-1638091
R-HSA-1638091
Reactome Name: Heparan sulfate/heparin (HS-GAG) metabolism
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605624
(
R-HSA-1660662
)
R-HSA-1605624
Reaction Name: Beta-galactosidase hydrolyses GM1 to GM2
See in Reactome
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-1606312
(
R-HSA-1660662
)
R-HSA-1606312
Reaction Name: Beta-galactosidase can also hydrolyse globosides to form cerebrosides
See in Reactome
R-HSA-1630306
(
R-HSA-2022857
)
R-HSA-1630306
Reaction Name: GLB1 hydrolyses a glycosaminoglycan
See in Reactome
R-HSA-2090079
(
R-HSA-2024096
)
R-HSA-2090079
Reaction Name: GLB1 hydrolyses linker chain(2)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
R-HSA-9638120
(
R-HSA-4085001
)
R-HSA-9638120
Reaction Name: NEU4 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
|
MONDO:0009262 |
GLB1 |
P16278 |
3.2.1.23
|
R-HSA-2022857
R-HSA-2024096
R-HSA-1660662
R-HSA-6798695
R-HSA-4085001
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-1638091
R-HSA-1638091
Reactome Name: Heparan sulfate/heparin (HS-GAG) metabolism
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605624
(
R-HSA-1660662
)
R-HSA-1605624
Reaction Name: Beta-galactosidase hydrolyses GM1 to GM2
See in Reactome
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-1606312
(
R-HSA-1660662
)
R-HSA-1606312
Reaction Name: Beta-galactosidase can also hydrolyse globosides to form cerebrosides
See in Reactome
R-HSA-1630306
(
R-HSA-2022857
)
R-HSA-1630306
Reaction Name: GLB1 hydrolyses a glycosaminoglycan
See in Reactome
R-HSA-2090079
(
R-HSA-2024096
)
R-HSA-2090079
Reaction Name: GLB1 hydrolyses linker chain(2)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
R-HSA-9638120
(
R-HSA-4085001
)
R-HSA-9638120
Reaction Name: NEU4 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
|
MONDO:0009265 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0009266 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0009267 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0009268 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0009591 |
ARSB |
P15848 |
3.1.6.12
|
R-HSA-6798695
R-HSA-2024101
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-71387
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606789
(
R-HSA-2024101
)
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-1793207
(
R-HSA-2024101
)
R-HSA-1793207
Reaction Name: ARSB hydrolyses C4S/C6S chains
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0009660 |
GLB1 |
P16278 |
3.2.1.23
|
R-HSA-2022857
R-HSA-2024096
R-HSA-1660662
R-HSA-6798695
R-HSA-4085001
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-1638091
R-HSA-1638091
Reactome Name: Heparan sulfate/heparin (HS-GAG) metabolism
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605624
(
R-HSA-1660662
)
R-HSA-1605624
Reaction Name: Beta-galactosidase hydrolyses GM1 to GM2
See in Reactome
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-1606312
(
R-HSA-1660662
)
R-HSA-1606312
Reaction Name: Beta-galactosidase can also hydrolyse globosides to form cerebrosides
See in Reactome
R-HSA-1630306
(
R-HSA-2022857
)
R-HSA-1630306
Reaction Name: GLB1 hydrolyses a glycosaminoglycan
See in Reactome
R-HSA-2090079
(
R-HSA-2024096
)
R-HSA-2090079
Reaction Name: GLB1 hydrolyses linker chain(2)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
R-HSA-9638120
(
R-HSA-4085001
)
R-HSA-9638120
Reaction Name: NEU4 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
|
MONDO:0009737 |
CTSA |
P10619 |
3.4.16.5
|
R-HSA-4085001
R-HSA-1660662
R-HSA-6798695
R-HSA-2132295
R-HSA-2132295
Reactome Name: MHC class II antigen presentation
See in Reactome
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168256
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-1280218
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-2130349
(
R-HSA-2132295
)
R-HSA-2130504
(
R-HSA-2132295
)
R-HSA-2130706
(
R-HSA-2132295
)
R-HSA-2130706
Reaction Name: MHC class II antigen processing
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-9638120
(
R-HSA-4085001
)
R-HSA-9638120
Reaction Name: NEU4 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
|
MONDO:0009756 |
SMPD1 |
P17405 |
3.1.4.12
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1605797
(
R-HSA-1660662
)
R-HSA-1605797
Reaction Name: Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome)
See in Reactome
|
MONDO:0010088 |
ARSA |
P15289 |
3.1.6.8
|
R-HSA-6798695
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606807
(
R-HSA-1660662
)
R-HSA-1606807
Reaction Name: Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside
See in Reactome
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-2248891
(
R-HSA-1660662
)
R-HSA-2248891
Reaction Name: Active ARSA translocates to the lysosome
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
|
MONDO:0010088 |
ARSB |
P15848 |
3.1.6.12
|
R-HSA-6798695
R-HSA-2024101
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-71387
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606789
(
R-HSA-2024101
)
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-1793207
(
R-HSA-2024101
)
R-HSA-1793207
Reaction Name: ARSB hydrolyses C4S/C6S chains
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0010088 |
SUMF1 |
Q8NBK3 |
1.8.3.7
|
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1614336
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614336
Reaction Name: SUMF2 binds SUMF1, inhibiting SUMF1-mediated activation of arylsulfatases
See in Reactome
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
|
MONDO:0010099 |
GM2A |
P17900 |
-
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605717
(
R-HSA-1660662
)
R-HSA-1605717
Reaction Name: Ganglioside GM2 activator presents GM2 to hexosaminidase for cleavage
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
|
MONDO:0010526 |
GLA |
P06280 |
3.2.1.22
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1605736
(
R-HSA-1660662
)
R-HSA-1605736
Reaction Name: Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
|
MONDO:0010555 |
ARSL |
P51690 |
3.1.6.1
|
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
|
MONDO:0010622 |
STS |
P08842 |
3.1.6.2
|
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1606839
(
R-HSA-1660662
)
R-HSA-1606839
Reaction Name: Steryl sulfatase hydrolyses sulfate from steroid sulfates
See in Reactome
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
|
MONDO:0011144 |
SMPD1 |
P17405 |
3.1.4.12
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1605797
(
R-HSA-1660662
)
R-HSA-1605797
Reaction Name: Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome)
See in Reactome
|
MONDO:0011871 |
SMPD1 |
P17405 |
3.1.4.12
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1605797
(
R-HSA-1660662
)
R-HSA-1605797
Reaction Name: Sphingomyelin phosphodiesterase (SMPD1) hydrolyses sphingomyelin to ceramide (lysosome)
See in Reactome
|
MONDO:0011945 |
GBA |
P04062 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-390471
R-HSA-390471
Reactome Name: Association of TriC/CCT with target proteins during biosynthesis
See in Reactome
R-HSA-1660662
|
R-HSA-391251
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-390466
R-HSA-390466
Reactome Name: Chaperonin-mediated protein folding
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1605591
(
R-HSA-1660662
)
R-HSA-1605591
Reaction Name: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
See in Reactome
R-HSA-390470
(
R-HSA-390471
)
R-HSA-390470
Reaction Name: Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)
See in Reactome
|
MONDO:0012213 |
B4GALNT1 |
Q00973 |
2.4.1.92
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-8856223
(
R-HSA-1660662
)
R-HSA-8856223
Reaction Name: B4GALNT1 dimer transfers GalNAc from UDP-GalNAc to GM3 and GD3 gangliosides
See in Reactome
|
MONDO:0013737 |
GBA2 |
Q9HCG7 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1861788
(
R-HSA-1660662
)
R-HSA-1861788
Reaction Name: Glucosylceramidase 2 cleaves the glucosidic bond of glucocerebroside to form ceramide (plasma membrane)
See in Reactome
|
MONDO:0015168 |
ASAH1 |
Q13510 |
3.5.1.23
3.5.1.-
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1606602
(
R-HSA-1660662
)
R-HSA-1606602
Reaction Name: Acid ceramidase hydrolyses ceramide into sphingosine and free fatty acid (lysosome)
See in Reactome
R-HSA-6798745
(
R-HSA-6798695
)
R-HSA-6798745
Reaction Name: Exocytosis of tertiary granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0016089 |
GALC |
P54803 |
3.2.1.46
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1606564
(
R-HSA-1660662
)
R-HSA-1606564
Reaction Name: Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide
See in Reactome
|
MONDO:0016090 |
GALC |
P54803 |
3.2.1.46
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1606564
(
R-HSA-1660662
)
R-HSA-1606564
Reaction Name: Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide
See in Reactome
|
MONDO:0016091 |
GALC |
P54803 |
3.2.1.46
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1606564
(
R-HSA-1660662
)
R-HSA-1606564
Reaction Name: Galactocerebrosidase cleaves the galactosyl bond of galactocerebroside to form ceramide
See in Reactome
|
MONDO:0016485 |
ARSG |
Q96EG1 |
3.1.6.15
3.1.6.1
|
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
|
MONDO:0017171 |
ARSB |
P15848 |
3.1.6.12
|
R-HSA-6798695
R-HSA-2024101
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-71387
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606789
(
R-HSA-2024101
)
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-1793207
(
R-HSA-2024101
)
R-HSA-1793207
Reaction Name: ARSB hydrolyses C4S/C6S chains
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0017172 |
ARSB |
P15848 |
3.1.6.12
|
R-HSA-6798695
R-HSA-2024101
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-71387
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
R-HSA-556833
R-HSA-1630316
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606789
(
R-HSA-2024101
)
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-1793207
(
R-HSA-2024101
)
R-HSA-1793207
Reaction Name: ARSB hydrolyses C4S/C6S chains
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-6800434
(
R-HSA-6798695
)
R-HSA-6800434
Reaction Name: Exocytosis of ficolin-rich granule lumen proteins
See in Reactome
|
MONDO:0017721 |
HEXB |
P07686 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-6798695
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-168249
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Immune System
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-9638075
(
R-HSA-2024101
)
R-HSA-9638075
Reaction Name: HEXB cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-9638076
(
R-HSA-2160916
)
R-HSA-9638076
Reaction Name: HEXB cleaves the terminal GalNAc from small HA fragments
See in Reactome
R-HSA-9638078
(
R-HSA-2022857
)
R-HSA-9638078
Reaction Name: HEXB cleaves the terminal GalNAc from keratan sulfate
See in Reactome
|
MONDO:0017722 |
HEXB |
P07686 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-6798695
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-168249
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Immune System
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-9638075
(
R-HSA-2024101
)
R-HSA-9638075
Reaction Name: HEXB cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-9638076
(
R-HSA-2160916
)
R-HSA-9638076
Reaction Name: HEXB cleaves the terminal GalNAc from small HA fragments
See in Reactome
R-HSA-9638078
(
R-HSA-2022857
)
R-HSA-9638078
Reaction Name: HEXB cleaves the terminal GalNAc from keratan sulfate
See in Reactome
|
MONDO:0017723 |
HEXB |
P07686 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-6798695
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-168256
R-HSA-168249
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Immune System
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
R-HSA-9638075
(
R-HSA-2024101
)
R-HSA-9638075
Reaction Name: HEXB cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-9638076
(
R-HSA-2160916
)
R-HSA-9638076
Reaction Name: HEXB cleaves the terminal GalNAc from small HA fragments
See in Reactome
R-HSA-9638078
(
R-HSA-2022857
)
R-HSA-9638078
Reaction Name: HEXB cleaves the terminal GalNAc from keratan sulfate
See in Reactome
|
MONDO:0017724 |
HEXA |
P06865 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
|
MONDO:0017725 |
HEXA |
P06865 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
|
MONDO:0017726 |
HEXA |
P06865 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
|
MONDO:0017728 |
HEXA |
P06865 |
3.2.1.52
|
R-HSA-2022857
R-HSA-2024101
R-HSA-1660662
R-HSA-2160916
R-HSA-2160916
Reactome Name: Hyaluronan uptake and degradation
See in Reactome
|
R-HSA-71387
R-HSA-1638074
R-HSA-1638074
Reactome Name: Keratan sulfate/keratin metabolism
See in Reactome
R-HSA-1793185
R-HSA-1793185
Reactome Name: Chondroitin sulfate/dermatan sulfate metabolism
See in Reactome
R-HSA-2142845
R-HSA-1430728
R-HSA-556833
R-HSA-1630316
R-HSA-428157
|
Metabolism
|
R-HSA-1605595
(
R-HSA-1660662
)
R-HSA-1605595
Reaction Name: Hexosaminidase A cleaves GalNAc from GM2 to form GM3
See in Reactome
R-HSA-1605632
(
R-HSA-1660662
)
R-HSA-1605632
Reaction Name: Both hexosaminidase A and B can cleave GalNAc from globoside
See in Reactome
R-HSA-1638053
(
R-HSA-2022857
)
R-HSA-1638053
Reaction Name: HEXA cleaves the terminal GalNAc from keratan sulfate
See in Reactome
R-HSA-2105001
(
R-HSA-2024101
)
R-HSA-2105001
Reaction Name: HEXA cleaves the terminal GalNAc from DS
See in Reactome
R-HSA-2162225
(
R-HSA-2160916
)
R-HSA-2162225
Reaction Name: HEXA cleaves the terminal GalNAc from small HA fragments
See in Reactome
|
MONDO:0017729 |
ARSA |
P15289 |
3.1.6.8
|
R-HSA-6798695
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606807
(
R-HSA-1660662
)
R-HSA-1606807
Reaction Name: Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside
See in Reactome
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-2248891
(
R-HSA-1660662
)
R-HSA-2248891
Reaction Name: Active ARSA translocates to the lysosome
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
|
MONDO:0017730 |
ARSA |
P15289 |
3.1.6.8
|
R-HSA-6798695
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-168256
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-168249
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1606807
(
R-HSA-1660662
)
R-HSA-1606807
Reaction Name: Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside
See in Reactome
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
R-HSA-2248891
(
R-HSA-1660662
)
R-HSA-2248891
Reaction Name: Active ARSA translocates to the lysosome
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
|
MONDO:0018129 |
GBA2 |
Q9HCG7 |
3.2.1.-
2.4.1.-
3.2.1.45
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1861788
(
R-HSA-1660662
)
R-HSA-1861788
Reaction Name: Glucosylceramidase 2 cleaves the glucosidic bond of glucocerebroside to form ceramide (plasma membrane)
See in Reactome
|
MONDO:0018418 |
ARSI |
Q5FYB1 |
3.1.6.-
|
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
|
MONDO:0019346 |
NEU1 |
Q99519 |
3.2.1.18
|
R-HSA-4085001
R-HSA-1660662
R-HSA-6798695
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798749
(
R-HSA-6798695
)
R-HSA-6798749
Reaction Name: Exocytosis of specific granule lumen proteins
See in Reactome
|
MONDO:0019681 |
NEU1 |
Q99519 |
3.2.1.18
|
R-HSA-4085001
R-HSA-1660662
R-HSA-6798695
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798749
(
R-HSA-6798695
)
R-HSA-6798749
Reaction Name: Exocytosis of specific granule lumen proteins
See in Reactome
|
MONDO:0019682 |
NEU1 |
Q99519 |
3.2.1.18
|
R-HSA-4085001
R-HSA-1660662
R-HSA-6798695
|
R-HSA-446219
R-HSA-446219
Reactome Name: Synthesis of substrates in N-glycan biosythesis
See in Reactome
R-HSA-446193
R-HSA-446193
Reactome Name: Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
See in Reactome
R-HSA-446203
R-HSA-446203
Reactome Name: Asparagine N-linked glycosylation
See in Reactome
R-HSA-168249
R-HSA-168256
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Immune System
Metabolism of proteins
Metabolism
|
R-HSA-1605724
(
R-HSA-1660662
)
R-HSA-1605724
Reaction Name: Neu5Ac is cleaved from GM3 by NEU1 and 4 to form a globoside (lysosomal lumen)
See in Reactome
R-HSA-4084999
(
R-HSA-4085001
)
R-HSA-4084999
Reaction Name: NEU1 hydrolyses Neu5Ac from glycoconjugates
See in Reactome
R-HSA-6798749
(
R-HSA-6798695
)
R-HSA-6798749
Reaction Name: Exocytosis of specific granule lumen proteins
See in Reactome
|
MONDO:0024573 |
GLA |
P06280 |
3.2.1.22
|
R-HSA-6798695
R-HSA-1660662
|
R-HSA-556833
R-HSA-168249
R-HSA-1430728
R-HSA-168256
R-HSA-428157
|
Metabolism
Immune System
|
R-HSA-1605736
(
R-HSA-1660662
)
R-HSA-1605736
Reaction Name: Alpha-galactosidase A removes a terminal galactose from alpha-D-galactoside oligomers
See in Reactome
R-HSA-6798751
(
R-HSA-6798695
)
R-HSA-6798751
Reaction Name: Exocytosis of azurophil granule lumen proteins
See in Reactome
|
MONDO:0029141 |
ARSG |
Q96EG1 |
3.1.6.15
3.1.6.1
|
R-HSA-1660662
R-HSA-1663150
R-HSA-1663150
Reactome Name: The activation of arylsulfatases
See in Reactome
|
R-HSA-163841
R-HSA-163841
Reactome Name: Gamma carboxylation, hypusine formation and arylsulfatase activation
See in Reactome
R-HSA-392499
R-HSA-428157
R-HSA-1430728
R-HSA-556833
R-HSA-597592
R-HSA-597592
Reactome Name: Post-translational protein modification
See in Reactome
|
Metabolism of proteins
Metabolism
|
R-HSA-1614362
(
R-HSA-1660662;
R-HSA-1663150
)
R-HSA-1614362
Reaction Name: SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
See in Reactome
|
MONDO:0032838 |
SMPD4 |
Q9NXE4 |
3.1.4.12
|
R-HSA-1660662
|
R-HSA-556833
R-HSA-428157
R-HSA-1430728
|
Metabolism
|
R-HSA-1606288
(
R-HSA-1660662
)
R-HSA-1606288
Reaction Name: Sphingomyelin phosphodiesterase 4 (SMPD4) hydrolyses sphingomyelin to ceramide (ER membrane)
See in Reactome
|