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
ABCD1 |
P33897 |
3.1.2.-
7.6.2.-
|
R-HSA-1369062
R-HSA-1369062
Reactome Name: ABC transporters in lipid homeostasis
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-9603798
R-HSA-9603798
Reactome Name: Class I peroxisomal membrane protein import
See in Reactome
R-HSA-2046105
|
R-HSA-9609507
R-HSA-382556
R-HSA-382556
Reactome Name: ABC-family proteins mediated transport
See in Reactome
R-HSA-390918
R-HSA-382551
R-HSA-1430728
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-556833
R-HSA-8978868
|
Protein localization
Transport of small molecules
Metabolism
|
R-HSA-2046087
(
R-HSA-2046106
)
R-HSA-2046087
Reaction Name: Translocation of tetracosahexaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-2046093
(
R-HSA-2046105
)
R-HSA-2046093
Reaction Name: Translocation of tetracosapentaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-382575
(
R-HSA-1369062
)
R-HSA-382575
Reaction Name: ABCD1-3 dimers transfer LCFAs from cytosol to peroxisomal matrix
See in Reactome
R-HSA-382613
(
R-HSA-1369062
)
R-HSA-382613
Reaction Name: PEX-19 docks ABCD1/D2/D3 to peroximal membrane
See in Reactome
R-HSA-390393
(
R-HSA-390247
)
R-HSA-390393
Reaction Name: Peroxisomal uptake of very long-chain fatty acyl CoA
See in Reactome
R-HSA-9603775
(
R-HSA-9603798
)
R-HSA-9603775
Reaction Name: PEX3:PEX19:class I PMP dissociates
See in Reactome
R-HSA-9603784
(
R-HSA-9603798
)
R-HSA-9603804
(
R-HSA-9603798
)
R-HSA-9603804
Reaction Name: PEX19 binds class I peroxisomal membrane proteins
See in Reactome
|
MONDO:0009300 |
HSD17B4 |
P51659 |
1.1.1.n12
4.2.1.119
4.2.1.107
|
R-HSA-389887
R-HSA-389887
Reactome Name: Beta-oxidation of pristanoyl-CoA
See in Reactome
R-HSA-193368
R-HSA-193368
Reactome Name: Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-9033500
R-HSA-9033500
Reactome Name: TYSND1 cleaves peroxisomal proteins
See in Reactome
R-HSA-9033241
|
R-HSA-194068
R-HSA-194068
Reactome Name: Bile acid and bile salt metabolism
See in Reactome
R-HSA-9609507
R-HSA-390918
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-1430728
R-HSA-8978868
R-HSA-192105
R-HSA-192105
Reactome Name: Synthesis of bile acids and bile salts
See in Reactome
R-HSA-556833
R-HSA-8957322
|
Protein localization
Metabolism
|
R-HSA-192331
(
R-HSA-193368
)
R-HSA-192331
Reaction Name: 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) is hydrated to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA
See in Reactome
R-HSA-193455
(
R-HSA-193368
)
R-HSA-193455
Reaction Name: (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoA
See in Reactome
R-HSA-193508
(
R-HSA-193368
)
R-HSA-193508
Reaction Name: (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoA
See in Reactome
R-HSA-193535
(
R-HSA-193368
)
R-HSA-193535
Reaction Name: 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA is hydrated to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA
See in Reactome
R-HSA-2066778
(
R-HSA-2046106
)
R-HSA-2066778
Reaction Name: Hydration of delta2-tetracosaheptaenoyl-CoA to 3-hydroxy tetracosahexaenoyl-CoA
See in Reactome
R-HSA-2066780
(
R-HSA-2046106
)
R-HSA-2066780
Reaction Name: Dehydrogenation of 3-hydroxy tetracosahexaenoyl-CoA
See in Reactome
R-HSA-389986
(
R-HSA-389887
)
R-HSA-389986
Reaction Name: trans-2,3-dehydropristanoyl-CoA + H2O => 3-hydroxypristanoyl-CoA
See in Reactome
R-HSA-389995
(
R-HSA-389887
)
R-HSA-389995
Reaction Name: 3-hydroxypristanoyl-CoA + NAD+ => 3-ketoxypristanoyl-CoA + NADH + H+
See in Reactome
R-HSA-390251
(
R-HSA-390247
)
R-HSA-390251
Reaction Name: HSD17B4 dehydrogenates 3-hydroxyhexacosanoyl-CoA
See in Reactome
R-HSA-390252
(
R-HSA-390247
)
R-HSA-390252
Reaction Name: HSD17B4 hydrates trans-2,3-dehydrohexacosanoyl-CoA
See in Reactome
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
Reaction Name: PEX5S,L binds cargo proteins containing PTS1
See in Reactome
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
Reaction Name: Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix
See in Reactome
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
Reaction Name: PEX5S,L:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module)
See in Reactome
R-HSA-9033530
(
R-HSA-9033241;
R-HSA-9033500
)
|
MONDO:0009410 |
ABCD1 |
P33897 |
3.1.2.-
7.6.2.-
|
R-HSA-1369062
R-HSA-1369062
Reactome Name: ABC transporters in lipid homeostasis
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-9603798
R-HSA-9603798
Reactome Name: Class I peroxisomal membrane protein import
See in Reactome
R-HSA-2046105
|
R-HSA-9609507
R-HSA-382556
R-HSA-382556
Reactome Name: ABC-family proteins mediated transport
See in Reactome
R-HSA-390918
R-HSA-382551
R-HSA-1430728
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-556833
R-HSA-8978868
|
Protein localization
Transport of small molecules
Metabolism
|
R-HSA-2046087
(
R-HSA-2046106
)
R-HSA-2046087
Reaction Name: Translocation of tetracosahexaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-2046093
(
R-HSA-2046105
)
R-HSA-2046093
Reaction Name: Translocation of tetracosapentaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-382575
(
R-HSA-1369062
)
R-HSA-382575
Reaction Name: ABCD1-3 dimers transfer LCFAs from cytosol to peroxisomal matrix
See in Reactome
R-HSA-382613
(
R-HSA-1369062
)
R-HSA-382613
Reaction Name: PEX-19 docks ABCD1/D2/D3 to peroximal membrane
See in Reactome
R-HSA-390393
(
R-HSA-390247
)
R-HSA-390393
Reaction Name: Peroxisomal uptake of very long-chain fatty acyl CoA
See in Reactome
R-HSA-9603775
(
R-HSA-9603798
)
R-HSA-9603775
Reaction Name: PEX3:PEX19:class I PMP dissociates
See in Reactome
R-HSA-9603784
(
R-HSA-9603798
)
R-HSA-9603804
(
R-HSA-9603798
)
R-HSA-9603804
Reaction Name: PEX19 binds class I peroxisomal membrane proteins
See in Reactome
|
MONDO:0009855 |
HSD17B4 |
P51659 |
1.1.1.n12
4.2.1.119
4.2.1.107
|
R-HSA-389887
R-HSA-389887
Reactome Name: Beta-oxidation of pristanoyl-CoA
See in Reactome
R-HSA-193368
R-HSA-193368
Reactome Name: Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-9033500
R-HSA-9033500
Reactome Name: TYSND1 cleaves peroxisomal proteins
See in Reactome
R-HSA-9033241
|
R-HSA-194068
R-HSA-194068
Reactome Name: Bile acid and bile salt metabolism
See in Reactome
R-HSA-9609507
R-HSA-390918
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-1430728
R-HSA-8978868
R-HSA-192105
R-HSA-192105
Reactome Name: Synthesis of bile acids and bile salts
See in Reactome
R-HSA-556833
R-HSA-8957322
|
Protein localization
Metabolism
|
R-HSA-192331
(
R-HSA-193368
)
R-HSA-192331
Reaction Name: 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-enoyl-CoA (THCA-CoA) is hydrated to (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA
See in Reactome
R-HSA-193455
(
R-HSA-193368
)
R-HSA-193455
Reaction Name: (24R, 25R) 3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha,12alpha-trihydroxy-5beta-cholest-24-one-CoA
See in Reactome
R-HSA-193508
(
R-HSA-193368
)
R-HSA-193508
Reaction Name: (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA is oxidized to 3alpha,7alpha-dihydroxy-5beta-cholest-24-one-CoA
See in Reactome
R-HSA-193535
(
R-HSA-193368
)
R-HSA-193535
Reaction Name: 25(S) 3alpha,7alpha-dihydroxy-5beta-cholest-24-enoyl-CoA is hydrated to (24R, 25R) 3alpha,7alpha,24-trihydroxy-5beta-cholestanoyl-CoA
See in Reactome
R-HSA-2066778
(
R-HSA-2046106
)
R-HSA-2066778
Reaction Name: Hydration of delta2-tetracosaheptaenoyl-CoA to 3-hydroxy tetracosahexaenoyl-CoA
See in Reactome
R-HSA-2066780
(
R-HSA-2046106
)
R-HSA-2066780
Reaction Name: Dehydrogenation of 3-hydroxy tetracosahexaenoyl-CoA
See in Reactome
R-HSA-389986
(
R-HSA-389887
)
R-HSA-389986
Reaction Name: trans-2,3-dehydropristanoyl-CoA + H2O => 3-hydroxypristanoyl-CoA
See in Reactome
R-HSA-389995
(
R-HSA-389887
)
R-HSA-389995
Reaction Name: 3-hydroxypristanoyl-CoA + NAD+ => 3-ketoxypristanoyl-CoA + NADH + H+
See in Reactome
R-HSA-390251
(
R-HSA-390247
)
R-HSA-390251
Reaction Name: HSD17B4 dehydrogenates 3-hydroxyhexacosanoyl-CoA
See in Reactome
R-HSA-390252
(
R-HSA-390247
)
R-HSA-390252
Reaction Name: HSD17B4 hydrates trans-2,3-dehydrohexacosanoyl-CoA
See in Reactome
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
Reaction Name: PEX5S,L binds cargo proteins containing PTS1
See in Reactome
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
Reaction Name: Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix
See in Reactome
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
Reaction Name: PEX5S,L:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module)
See in Reactome
R-HSA-9033530
(
R-HSA-9033241;
R-HSA-9033500
)
|
MONDO:0009919 |
ACOX1 |
Q15067 |
1.3.3.6
|
R-HSA-1989781
R-HSA-1989781
Reactome Name: PPARA activates gene expression
See in Reactome
R-HSA-9033500
R-HSA-9033500
Reactome Name: TYSND1 cleaves peroxisomal proteins
See in Reactome
R-HSA-9033241
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
|
R-HSA-9609507
R-HSA-400206
R-HSA-400206
Reactome Name: Regulation of lipid metabolism by PPARalpha
See in Reactome
R-HSA-390918
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-1430728
R-HSA-8978868
R-HSA-556833
|
Protein localization
Metabolism
|
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
Reaction Name: PEX5S,L binds cargo proteins containing PTS1
See in Reactome
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
Reaction Name: Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix
See in Reactome
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
Reaction Name: PEX5S,L:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module)
See in Reactome
R-HSA-9033515
(
R-HSA-9033241;
R-HSA-9033500
)
|
MONDO:0010247 |
ABCD1 |
P33897 |
3.1.2.-
7.6.2.-
|
R-HSA-1369062
R-HSA-1369062
Reactome Name: ABC transporters in lipid homeostasis
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-9603798
R-HSA-9603798
Reactome Name: Class I peroxisomal membrane protein import
See in Reactome
R-HSA-2046105
|
R-HSA-9609507
R-HSA-382556
R-HSA-382556
Reactome Name: ABC-family proteins mediated transport
See in Reactome
R-HSA-390918
R-HSA-382551
R-HSA-1430728
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-556833
R-HSA-8978868
|
Protein localization
Transport of small molecules
Metabolism
|
R-HSA-2046087
(
R-HSA-2046106
)
R-HSA-2046087
Reaction Name: Translocation of tetracosahexaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-2046093
(
R-HSA-2046105
)
R-HSA-2046093
Reaction Name: Translocation of tetracosapentaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-382575
(
R-HSA-1369062
)
R-HSA-382575
Reaction Name: ABCD1-3 dimers transfer LCFAs from cytosol to peroxisomal matrix
See in Reactome
R-HSA-382613
(
R-HSA-1369062
)
R-HSA-382613
Reaction Name: PEX-19 docks ABCD1/D2/D3 to peroximal membrane
See in Reactome
R-HSA-390393
(
R-HSA-390247
)
R-HSA-390393
Reaction Name: Peroxisomal uptake of very long-chain fatty acyl CoA
See in Reactome
R-HSA-9603775
(
R-HSA-9603798
)
R-HSA-9603775
Reaction Name: PEX3:PEX19:class I PMP dissociates
See in Reactome
R-HSA-9603784
(
R-HSA-9603798
)
R-HSA-9603804
(
R-HSA-9603798
)
R-HSA-9603804
Reaction Name: PEX19 binds class I peroxisomal membrane proteins
See in Reactome
|
MONDO:0013391 |
SCP2 |
P22307 |
2.3.1.176
2.3.1.16
2.3.1.155
|
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-389887
R-HSA-389887
Reactome Name: Beta-oxidation of pristanoyl-CoA
See in Reactome
R-HSA-193368
R-HSA-193368
Reactome Name: Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol
See in Reactome
R-HSA-9033241
R-HSA-9033500
R-HSA-9033500
Reactome Name: TYSND1 cleaves peroxisomal proteins
See in Reactome
|
R-HSA-194068
R-HSA-194068
Reactome Name: Bile acid and bile salt metabolism
See in Reactome
R-HSA-9609507
R-HSA-390918
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-1430728
R-HSA-8978868
R-HSA-192105
R-HSA-192105
Reactome Name: Synthesis of bile acids and bile salts
See in Reactome
R-HSA-556833
R-HSA-8957322
|
Protein localization
Metabolism
|
R-HSA-2066781
(
R-HSA-2046106
)
R-HSA-2066781
Reaction Name: Formation of DHA-CoA catalysed by sterol carrier protein X (SCPx)
See in Reactome
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
Reaction Name: PEX5S,L binds cargo proteins containing PTS1
See in Reactome
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
Reaction Name: Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix
See in Reactome
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
Reaction Name: PEX5S,L:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module)
See in Reactome
R-HSA-9033524
(
R-HSA-9033241;
R-HSA-9033500
)
|
MONDO:0014417 |
ELOVL5 |
Q9NYP7 |
2.3.1.199
|
R-HSA-75876
R-HSA-75876
Reactome Name: Synthesis of very long-chain fatty acyl-CoAs
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-2046105
|
R-HSA-556833
R-HSA-75105
R-HSA-1430728
R-HSA-8978868
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
|
Metabolism
|
R-HSA-2046083
(
R-HSA-2046105
)
R-HSA-2046083
Reaction Name: Elongation of arachidonyl-CoA to docosatetraenoyl-CoA
See in Reactome
R-HSA-2046088
(
R-HSA-2046106
)
R-HSA-2046088
Reaction Name: Elongation of stearidonoyl-CoA to eicosatetraenoyl-CoA
See in Reactome
R-HSA-2046094
(
R-HSA-2046105
)
R-HSA-2046094
Reaction Name: Elongation of gamma-lenolenoyl-CoA to dihomo-gamma-lenolenoyl-CoA
See in Reactome
R-HSA-2046100
(
R-HSA-2046106
)
R-HSA-2046100
Reaction Name: Elongation of eicosapentaenoyl-CoA to docosapentaenoyl-CoA
See in Reactome
R-HSA-548800
(
R-HSA-75876
)
R-HSA-548800
Reaction Name: ELOVL1,2,3,5 elongate AA-CoA and Mal-CoA to 3ODCT-CoA
See in Reactome
|
MONDO:0015339 |
ABCD1 |
P33897 |
3.1.2.-
7.6.2.-
|
R-HSA-1369062
R-HSA-1369062
Reactome Name: ABC transporters in lipid homeostasis
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-9603798
R-HSA-9603798
Reactome Name: Class I peroxisomal membrane protein import
See in Reactome
R-HSA-2046105
|
R-HSA-9609507
R-HSA-382556
R-HSA-382556
Reactome Name: ABC-family proteins mediated transport
See in Reactome
R-HSA-390918
R-HSA-382551
R-HSA-1430728
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-556833
R-HSA-8978868
|
Protein localization
Transport of small molecules
Metabolism
|
R-HSA-2046087
(
R-HSA-2046106
)
R-HSA-2046087
Reaction Name: Translocation of tetracosahexaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-2046093
(
R-HSA-2046105
)
R-HSA-2046093
Reaction Name: Translocation of tetracosapentaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-382575
(
R-HSA-1369062
)
R-HSA-382575
Reaction Name: ABCD1-3 dimers transfer LCFAs from cytosol to peroxisomal matrix
See in Reactome
R-HSA-382613
(
R-HSA-1369062
)
R-HSA-382613
Reaction Name: PEX-19 docks ABCD1/D2/D3 to peroximal membrane
See in Reactome
R-HSA-390393
(
R-HSA-390247
)
R-HSA-390393
Reaction Name: Peroxisomal uptake of very long-chain fatty acyl CoA
See in Reactome
R-HSA-9603775
(
R-HSA-9603798
)
R-HSA-9603775
Reaction Name: PEX3:PEX19:class I PMP dissociates
See in Reactome
R-HSA-9603784
(
R-HSA-9603798
)
R-HSA-9603804
(
R-HSA-9603798
)
R-HSA-9603804
Reaction Name: PEX19 binds class I peroxisomal membrane proteins
See in Reactome
|
MONDO:0018309 |
ABCD1 |
P33897 |
3.1.2.-
7.6.2.-
|
R-HSA-1369062
R-HSA-1369062
Reactome Name: ABC transporters in lipid homeostasis
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-9603798
R-HSA-9603798
Reactome Name: Class I peroxisomal membrane protein import
See in Reactome
R-HSA-2046105
|
R-HSA-9609507
R-HSA-382556
R-HSA-382556
Reactome Name: ABC-family proteins mediated transport
See in Reactome
R-HSA-390918
R-HSA-382551
R-HSA-1430728
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-556833
R-HSA-8978868
|
Protein localization
Transport of small molecules
Metabolism
|
R-HSA-2046087
(
R-HSA-2046106
)
R-HSA-2046087
Reaction Name: Translocation of tetracosahexaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-2046093
(
R-HSA-2046105
)
R-HSA-2046093
Reaction Name: Translocation of tetracosapentaenoyl-CoA to peroxisomes
See in Reactome
R-HSA-382575
(
R-HSA-1369062
)
R-HSA-382575
Reaction Name: ABCD1-3 dimers transfer LCFAs from cytosol to peroxisomal matrix
See in Reactome
R-HSA-382613
(
R-HSA-1369062
)
R-HSA-382613
Reaction Name: PEX-19 docks ABCD1/D2/D3 to peroximal membrane
See in Reactome
R-HSA-390393
(
R-HSA-390247
)
R-HSA-390393
Reaction Name: Peroxisomal uptake of very long-chain fatty acyl CoA
See in Reactome
R-HSA-9603775
(
R-HSA-9603798
)
R-HSA-9603775
Reaction Name: PEX3:PEX19:class I PMP dissociates
See in Reactome
R-HSA-9603784
(
R-HSA-9603798
)
R-HSA-9603804
(
R-HSA-9603798
)
R-HSA-9603804
Reaction Name: PEX19 binds class I peroxisomal membrane proteins
See in Reactome
|
MONDO:0030073 |
ACOX1 |
Q15067 |
1.3.3.6
|
R-HSA-1989781
R-HSA-1989781
Reactome Name: PPARA activates gene expression
See in Reactome
R-HSA-9033500
R-HSA-9033500
Reactome Name: TYSND1 cleaves peroxisomal proteins
See in Reactome
R-HSA-9033241
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-390247
R-HSA-390247
Reactome Name: Beta-oxidation of very long chain fatty acids
See in Reactome
|
R-HSA-9609507
R-HSA-400206
R-HSA-400206
Reactome Name: Regulation of lipid metabolism by PPARalpha
See in Reactome
R-HSA-390918
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
R-HSA-1430728
R-HSA-8978868
R-HSA-556833
|
Protein localization
Metabolism
|
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
(
R-HSA-9033241
)
R-HSA-9033233
Reaction Name: PEX5S,L binds cargo proteins containing PTS1
See in Reactome
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
(
R-HSA-9033241
)
R-HSA-9033235
Reaction Name: Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix
See in Reactome
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
(
R-HSA-9033241
)
R-HSA-9033236
Reaction Name: PEX5S,L:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module)
See in Reactome
R-HSA-9033515
(
R-HSA-9033241;
R-HSA-9033500
)
|
MONDO:0032798 |
ELOVL1 |
Q9BW60 |
2.3.1.199
|
R-HSA-75876
R-HSA-75876
Reactome Name: Synthesis of very long-chain fatty acyl-CoAs
See in Reactome
R-HSA-2046106
R-HSA-2046106
Reactome Name: alpha-linolenic acid (ALA) metabolism
See in Reactome
R-HSA-2046105
|
R-HSA-556833
R-HSA-75105
R-HSA-1430728
R-HSA-8978868
R-HSA-2046104
R-HSA-2046104
Reactome Name: alpha-linolenic (omega3) and linoleic (omega6) acid metabolism
See in Reactome
|
Metabolism
|
R-HSA-2046088
(
R-HSA-2046106
)
R-HSA-2046088
Reaction Name: Elongation of stearidonoyl-CoA to eicosatetraenoyl-CoA
See in Reactome
R-HSA-2046094
(
R-HSA-2046105
)
R-HSA-2046094
Reaction Name: Elongation of gamma-lenolenoyl-CoA to dihomo-gamma-lenolenoyl-CoA
See in Reactome
R-HSA-548800
(
R-HSA-75876
)
R-HSA-548800
Reaction Name: ELOVL1,2,3,5 elongate AA-CoA and Mal-CoA to 3ODCT-CoA
See in Reactome
R-HSA-548830
(
R-HSA-75876
)
R-HSA-548830
Reaction Name: ELOVL1,4 elongate TCS-CoA and Mal-CoA to 3OHC-CoA
See in Reactome
|