DAR - Bologna Biocomputing Group

Search results (Reactome ID: R-HSA-389661)

DiseaseID	Gene	UniProt	EC numbers	Reactomes	Expanded Reactomes	Reactome roots	Reactions
MONDO:0001567	AGXT	P21549	2.6.1.44 2.6.1.51	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-9033241 R-HSA-9033241 Reactome Name: Peroxisomal protein import See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-389684 ( R-HSA-389661 ) R-HSA-389684 Reaction Name: glyoxylate + alanine => glycine + pyruvate [peroxisome] See in Reactome R-HSA-9033233 ( R-HSA-9033241 ) R-HSA-9033233 Reaction Name: PEX5S See in Reactome 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 Reaction Name: Cargo of PEX5S See in Reactome 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 Reaction Name: PEX5S See in Reactome 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
MONDO:0005090	DAO	P14920	1.4.3.3	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-9033241 R-HSA-9033241 Reactome Name: Peroxisomal protein import See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-389821 ( R-HSA-389661 ) R-HSA-389821 Reaction Name: glycine + O2 => glyoxylate + H2O2 + NH4+ See in Reactome R-HSA-9033233 ( R-HSA-9033241 ) R-HSA-9033233 Reaction Name: PEX5S See in Reactome 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 Reaction Name: Cargo of PEX5S See in Reactome 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 Reaction Name: PEX5S See in Reactome 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
MONDO:0005144	DAO	P14920	1.4.3.3	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-9033241 R-HSA-9033241 Reactome Name: Peroxisomal protein import See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-389821 ( R-HSA-389661 ) R-HSA-389821 Reaction Name: glycine + O2 => glyoxylate + H2O2 + NH4+ See in Reactome R-HSA-9033233 ( R-HSA-9033241 ) R-HSA-9033233 Reaction Name: PEX5S See in Reactome 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 Reaction Name: Cargo of PEX5S See in Reactome 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 Reaction Name: PEX5S See in Reactome 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
MONDO:0008171	AGXT	P21549	2.6.1.44 2.6.1.51	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-9033241 R-HSA-9033241 Reactome Name: Peroxisomal protein import See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-389684 ( R-HSA-389661 ) R-HSA-389684 Reaction Name: glyoxylate + alanine => glycine + pyruvate [peroxisome] See in Reactome R-HSA-9033233 ( R-HSA-9033241 ) R-HSA-9033233 Reaction Name: PEX5S See in Reactome 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 Reaction Name: Cargo of PEX5S See in Reactome 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 Reaction Name: PEX5S See in Reactome 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
MONDO:0008759	OGDH	Q02218	1.2.4.2	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-71403 R-HSA-71403 Reactome Name: Citric acid cycle (TCA cycle) See in Reactome R-HSA-71064 R-HSA-71064 Reactome Name: Lysine catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71037 ( R-HSA-71064 ) R-HSA-71037 Reaction Name: alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+ See in Reactome R-HSA-71401 ( R-HSA-71403 ) R-HSA-71401 Reaction Name: alpha-ketoglutarate + CoASH + NAD+ => succinyl-CoA + CO2 + NADH + H+ See in Reactome
MONDO:0008774	DHTKD1	Q96HY7	1.2.4.2	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome
MONDO:0009401	ALDH4A1	P30038	1.2.1.88	R-HSA-70688 R-HSA-70688 Reactome Name: Proline catabolism See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6784399 ( R-HSA-389661 ) R-HSA-6784399 Reaction Name: ALDH4A1 dimer dehydrogenates 4-OH-L-glutamate semialdehyde to 4-OH-L-glutamate See in Reactome R-HSA-70679 ( R-HSA-70688 ) R-HSA-70679 Reaction Name: ALDH4A1 oxidises L-GluSS to Glu See in Reactome
MONDO:0009502	DLAT	P10515	2.3.1.12	R-HSA-204174 R-HSA-204174 Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70268 R-HSA-70268 Reactome Name: Pyruvate metabolism See in Reactome R-HSA-5362517 R-HSA-5362517 Reactome Name: Signaling by Retinoic Acid See in Reactome	R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-9006931 R-HSA-9006931 Reactome Name: Signaling by Nuclear Receptors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-203946 ( R-HSA-204174; R-HSA-5362517; R-HSA-70268 ) R-HSA-203946 Reaction Name: PDK isoforms phosphorylate lipo-PDH See in Reactome R-HSA-204169 ( R-HSA-204174; R-HSA-70268 ) R-HSA-204169 Reaction Name: PDP dephosphorylates p-lipo-PDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71397 ( R-HSA-70268 ) R-HSA-71397 Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA See in Reactome
MONDO:0009529	DLD	P09622	1.8.1.4	R-HSA-71403 R-HSA-71403 Reactome Name: Citric acid cycle (TCA cycle) See in Reactome R-HSA-204174 R-HSA-204174 Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-70268 R-HSA-70268 Reactome Name: Pyruvate metabolism See in Reactome R-HSA-71064 R-HSA-71064 Reactome Name: Lysine catabolism See in Reactome R-HSA-6783984 R-HSA-6783984 Reactome Name: Glycine degradation See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-5362517 R-HSA-5362517 Reactome Name: Signaling by Retinoic Acid See in Reactome	R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-9006931 R-HSA-9006931 Reactome Name: Signaling by Nuclear Receptors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-203946 ( R-HSA-204174; R-HSA-5362517; R-HSA-70268 ) R-HSA-203946 Reaction Name: PDK isoforms phosphorylate lipo-PDH See in Reactome R-HSA-204169 ( R-HSA-204174; R-HSA-70268 ) R-HSA-204169 Reaction Name: PDP dephosphorylates p-lipo-PDH See in Reactome R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-5694018 ( R-HSA-389661; R-HSA-6783984 ) R-HSA-5694018 Reaction Name: DLD dimer:2xFAD oxidises GCSH:DHLL to GCSH:lipoate See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome R-HSA-71037 ( R-HSA-71064 ) R-HSA-71037 Reaction Name: alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+ See in Reactome R-HSA-71397 ( R-HSA-70268 ) R-HSA-71397 Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA See in Reactome R-HSA-71401 ( R-HSA-71403 ) R-HSA-71401 Reaction Name: alpha-ketoglutarate + CoASH + NAD+ => succinyl-CoA + CO2 + NADH + H+ See in Reactome
MONDO:0009776	PDHA2	P29803	1.2.4.1	R-HSA-204174 R-HSA-204174 Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70268 R-HSA-70268 Reactome Name: Pyruvate metabolism See in Reactome R-HSA-5362517 R-HSA-5362517 Reactome Name: Signaling by Retinoic Acid See in Reactome	R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-9006931 R-HSA-9006931 Reactome Name: Signaling by Nuclear Receptors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-203946 ( R-HSA-204174; R-HSA-5362517; R-HSA-70268 ) R-HSA-203946 Reaction Name: PDK isoforms phosphorylate lipo-PDH See in Reactome R-HSA-204169 ( R-HSA-204174; R-HSA-70268 ) R-HSA-204169 Reaction Name: PDP dephosphorylates p-lipo-PDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71397 ( R-HSA-70268 ) R-HSA-71397 Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA See in Reactome
MONDO:0009823	AGXT	P21549	2.6.1.44 2.6.1.51	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-9033241 R-HSA-9033241 Reactome Name: Peroxisomal protein import See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9609507 R-HSA-9609507 Reactome Name: Protein localization See in Reactome	Metabolism Protein localization	R-HSA-389684 ( R-HSA-389661 ) R-HSA-389684 Reaction Name: glyoxylate + alanine => glycine + pyruvate [peroxisome] See in Reactome R-HSA-9033233 ( R-HSA-9033241 ) R-HSA-9033233 Reaction Name: PEX5S See in Reactome 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 Reaction Name: Cargo of PEX5S See in Reactome 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 Reaction Name: PEX5S See in Reactome 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
MONDO:0009824	GRHPR	Q9UBQ7	1.1.1.81 1.1.1.79	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-389826 ( R-HSA-389661 ) R-HSA-389826 Reaction Name: glyoxylate + NADPH + H+ => glycolate + NADP+ See in Reactome
MONDO:0010717	PDHA1	P08559	1.2.4.1	R-HSA-204174 R-HSA-204174 Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70268 R-HSA-70268 Reactome Name: Pyruvate metabolism See in Reactome R-HSA-5362517 R-HSA-5362517 Reactome Name: Signaling by Retinoic Acid See in Reactome	R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-9006931 R-HSA-9006931 Reactome Name: Signaling by Nuclear Receptors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-203946 ( R-HSA-204174; R-HSA-5362517; R-HSA-70268 ) R-HSA-203946 Reaction Name: PDK isoforms phosphorylate lipo-PDH See in Reactome R-HSA-204169 ( R-HSA-204174; R-HSA-70268 ) R-HSA-204169 Reaction Name: PDP dephosphorylates p-lipo-PDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71397 ( R-HSA-70268 ) R-HSA-71397 Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA See in Reactome
MONDO:0011698	GNMT	Q14749	2.1.1.20	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-2408508 R-HSA-2408508 Reactome Name: Metabolism of ingested SeMet, Sec, MeSec into H2Se See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-2408522 R-HSA-2408522 Reactome Name: Selenoamino acid metabolism See in Reactome	Metabolism	R-HSA-2408544 ( R-HSA-2408508 ) R-HSA-2408544 Reaction Name: AdoSeMet is converted to AdeSeHCys by MetTrans(1) See in Reactome R-HSA-6798317 ( R-HSA-389661 ) R-HSA-6798317 Reaction Name: GNMT tetramer transfers methyl group from AdoMet to Gly to form AdoHyc and SARC See in Reactome
MONDO:0013327	HOGA1	Q86XE5	4.1.3.16	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6784423 ( R-HSA-389661 ) R-HSA-6784423 Reaction Name: HOGA1 tetramer aldol-cleaves 4-OH-2-oxoglutarate (HOG) to glyoxylate and pyruvate See in Reactome
MONDO:0013580	PDHB	P11177	1.2.4.1	R-HSA-204174 R-HSA-204174 Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70268 R-HSA-70268 Reactome Name: Pyruvate metabolism See in Reactome R-HSA-5362517 R-HSA-5362517 Reactome Name: Signaling by Retinoic Acid See in Reactome	R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-9006931 R-HSA-9006931 Reactome Name: Signaling by Nuclear Receptors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-203946 ( R-HSA-204174; R-HSA-5362517; R-HSA-70268 ) R-HSA-203946 Reaction Name: PDK isoforms phosphorylate lipo-PDH See in Reactome R-HSA-204169 ( R-HSA-204174; R-HSA-70268 ) R-HSA-204169 Reaction Name: PDP dephosphorylates p-lipo-PDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71397 ( R-HSA-70268 ) R-HSA-71397 Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA See in Reactome
MONDO:0013762	LIAS	O43766	2.8.1.8	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6793591 ( R-HSA-389661 ) R-HSA-6793591 Reaction Name: LIAS:2(4Fe-4S) transforms octanoyl-K107-GCSH to lipoyl-K107-GCSH See in Reactome
MONDO:0014012	DHTKD1	Q96HY7	1.2.4.2	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome
MONDO:0014576	LIPT1	Q9Y234	2.3.1.-	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome
MONDO:0016815	PDHA1	P08559	1.2.4.1	R-HSA-204174 R-HSA-204174 Reactome Name: Regulation of pyruvate dehydrogenase (PDH) complex See in Reactome R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70268 R-HSA-70268 Reactome Name: Pyruvate metabolism See in Reactome R-HSA-5362517 R-HSA-5362517 Reactome Name: Signaling by Retinoic Acid See in Reactome	R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-9006931 R-HSA-9006931 Reactome Name: Signaling by Nuclear Receptors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-203946 ( R-HSA-204174; R-HSA-5362517; R-HSA-70268 ) R-HSA-203946 Reaction Name: PDK isoforms phosphorylate lipo-PDH See in Reactome R-HSA-204169 ( R-HSA-204174; R-HSA-70268 ) R-HSA-204169 Reaction Name: PDP dephosphorylates p-lipo-PDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71397 ( R-HSA-70268 ) R-HSA-71397 Reaction Name: lipo-PDH decarboxylates PYR to Ac-CoA See in Reactome
MONDO:0016815	LIPT1	Q9Y234	2.3.1.-	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome
MONDO:0017051	DBT	P11182	2.3.1.168	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-9013407 R-HSA-9013407 Reactome Name: RHOH GTPase cycle See in Reactome	R-HSA-9012999 R-HSA-9012999 Reactome Name: RHO GTPase cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-194315 R-HSA-194315 Reactome Name: Signaling by Rho GTPases See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9716542 R-HSA-9716542 Reactome Name: Signaling by Rho GTPases, Miro GTPases and RHOBTB3 See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome R-HSA-9018758 ( R-HSA-9013407 ) R-HSA-9018758 Reaction Name: RHOH binds effectors at the plasma membrane See in Reactome
MONDO:0017051	BCKDHA	P12694	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017051	BCKDHB	P21953	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017052	DBT	P11182	2.3.1.168	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-9013407 R-HSA-9013407 Reactome Name: RHOH GTPase cycle See in Reactome	R-HSA-9012999 R-HSA-9012999 Reactome Name: RHO GTPase cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-194315 R-HSA-194315 Reactome Name: Signaling by Rho GTPases See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9716542 R-HSA-9716542 Reactome Name: Signaling by Rho GTPases, Miro GTPases and RHOBTB3 See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome R-HSA-9018758 ( R-HSA-9013407 ) R-HSA-9018758 Reaction Name: RHOH binds effectors at the plasma membrane See in Reactome
MONDO:0017052	BCKDHA	P12694	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017052	BCKDHB	P21953	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017053	DBT	P11182	2.3.1.168	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-9013407 R-HSA-9013407 Reactome Name: RHOH GTPase cycle See in Reactome	R-HSA-9012999 R-HSA-9012999 Reactome Name: RHO GTPase cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-194315 R-HSA-194315 Reactome Name: Signaling by Rho GTPases See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9716542 R-HSA-9716542 Reactome Name: Signaling by Rho GTPases, Miro GTPases and RHOBTB3 See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome R-HSA-9018758 ( R-HSA-9013407 ) R-HSA-9018758 Reaction Name: RHOH binds effectors at the plasma membrane See in Reactome
MONDO:0017053	BCKDHA	P12694	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017053	BCKDHB	P21953	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017054	DBT	P11182	2.3.1.168	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-9013407 R-HSA-9013407 Reactome Name: RHOH GTPase cycle See in Reactome	R-HSA-9012999 R-HSA-9012999 Reactome Name: RHO GTPase cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-194315 R-HSA-194315 Reactome Name: Signaling by Rho GTPases See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9716542 R-HSA-9716542 Reactome Name: Signaling by Rho GTPases, Miro GTPases and RHOBTB3 See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome R-HSA-9018758 ( R-HSA-9013407 ) R-HSA-9018758 Reaction Name: RHOH binds effectors at the plasma membrane See in Reactome
MONDO:0017054	BCKDHA	P12694	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017054	BCKDHB	P21953	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0017366	DLST	P36957	2.3.1.61	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-71403 R-HSA-71403 Reactome Name: Citric acid cycle (TCA cycle) See in Reactome R-HSA-71064 R-HSA-71064 Reactome Name: Lysine catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71037 ( R-HSA-71064 ) R-HSA-71037 Reaction Name: alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+ See in Reactome R-HSA-71401 ( R-HSA-71403 ) R-HSA-71401 Reaction Name: alpha-ketoglutarate + CoASH + NAD+ => succinyl-CoA + CO2 + NADH + H+ See in Reactome
MONDO:0023691	BCKDHA	P12694	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0023692	BCKDHB	P21953	1.2.4.4	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome
MONDO:0023693	DBT	P11182	2.3.1.168	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-9013407 R-HSA-9013407 Reactome Name: RHOH GTPase cycle See in Reactome	R-HSA-9012999 R-HSA-9012999 Reactome Name: RHO GTPase cycle See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-194315 R-HSA-194315 Reactome Name: Signaling by Rho GTPases See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-9716542 R-HSA-9716542 Reactome Name: Signaling by Rho GTPases, Miro GTPases and RHOBTB3 See in Reactome R-HSA-162582 R-HSA-162582 Reactome Name: Signal Transduction See in Reactome	Metabolism Signal Transduction	R-HSA-5693148 ( R-HSA-70895 ) R-HSA-5693148 Reaction Name: BCKDK phosphorylates BCKDH See in Reactome R-HSA-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-70713 ( R-HSA-70895 ) R-HSA-70713 Reaction Name: BCKDH transfers CoA group from CoA-SH to BCAAs See in Reactome R-HSA-9018758 ( R-HSA-9013407 ) R-HSA-9018758 Reaction Name: RHOH binds effectors at the plasma membrane See in Reactome
MONDO:0032771	DLST	P36957	2.3.1.61	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-71403 R-HSA-71403 Reactome Name: Citric acid cycle (TCA cycle) See in Reactome R-HSA-71064 R-HSA-71064 Reactome Name: Lysine catabolism See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-71406 R-HSA-71406 Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-1428517 R-HSA-1428517 Reactome Name: The citric acid (TCA) cycle and respiratory electron transport See in Reactome	Metabolism	R-HSA-6792572 ( R-HSA-389661 ) R-HSA-6792572 Reaction Name: LIPT1 transfers lipoyl group from lipoyl-GCSH to DHs See in Reactome R-HSA-71037 ( R-HSA-71064 ) R-HSA-71037 Reaction Name: alpha-ketoadipate + CoASH + NAD+ => glutaryl-CoA + CO2 + NADH + H+ See in Reactome R-HSA-71401 ( R-HSA-71403 ) R-HSA-71401 Reaction Name: alpha-ketoglutarate + CoASH + NAD+ => succinyl-CoA + CO2 + NADH + H+ See in Reactome
MONDO:0032880	GOT2	P00505	2.6.1.7 2.6.1.1	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome R-HSA-8964539 R-HSA-8964539 Reactome Name: Glutamate and glutamine metabolism See in Reactome R-HSA-1614558 R-HSA-1614558 Reactome Name: Degradation of cysteine and homocysteine See in Reactome R-HSA-8963693 R-HSA-8963693 Reactome Name: Aspartate and asparagine metabolism See in Reactome R-HSA-70263 R-HSA-70263 Reactome Name: Gluconeogenesis See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1614635 R-HSA-1614635 Reactome Name: Sulfur amino acid metabolism See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-70326 R-HSA-70326 Reactome Name: Glucose metabolism See in Reactome R-HSA-71387 R-HSA-71387 Reactome Name: Metabolism of carbohydrates See in Reactome	Metabolism	R-HSA-6784393 ( R-HSA-389661 ) R-HSA-6784393 Reaction Name: PXLP-K279-GOT2 dimer transaminates 4-OH-L-glutamate to 4-hydroxy-2-oxoglutarate (HOG) See in Reactome R-HSA-70596 ( R-HSA-8963693 ) R-HSA-70596 Reaction Name: GOT2 dimer deaminates L-Asp See in Reactome R-HSA-70613 ( R-HSA-70263; R-HSA-8964539 ) R-HSA-70613 Reaction Name: oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT2] See in Reactome R-HSA-9012597 ( R-HSA-1614558 ) R-HSA-9012597 Reaction Name: GOT2 dimer transfers amino group from L-Cys to 2OG to form 3MPYR and Glu See in Reactome
MONDO:0060562	LIPT2	A6NK58	2.3.1.181	R-HSA-389661 R-HSA-389661 Reactome Name: Glyoxylate metabolism and glycine degradation See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome	Metabolism	R-HSA-6793590 ( R-HSA-389661 ) R-HSA-6793590 Reaction Name: LIPT2 transfers octanoyl group to GCSH See in Reactome

Number of enzymes:	21
Number of disease:	31
Number of reactomes:	16
Number of reactions:	28

Search summary (Reactome ID: R-HSA-389661)

Search results (Reactome ID: R-HSA-389661)

MONDO:0001567

R-HSA-389661

R-HSA-9033241

R-HSA-71291

R-HSA-1430728

R-HSA-9609507

R-HSA-389684

R-HSA-9033233

R-HSA-9033233

R-HSA-9033235

R-HSA-9033235

R-HSA-9033236

R-HSA-9033236

MONDO:0005090

R-HSA-389661

R-HSA-9033241

R-HSA-71291

R-HSA-1430728

R-HSA-9609507

R-HSA-389821

R-HSA-9033233

R-HSA-9033233

R-HSA-9033235

R-HSA-9033235

R-HSA-9033236

R-HSA-9033236

MONDO:0005144

R-HSA-389661

R-HSA-9033241

R-HSA-71291

R-HSA-1430728

R-HSA-9609507

R-HSA-389821

R-HSA-9033233

R-HSA-9033233

R-HSA-9033235

R-HSA-9033235

R-HSA-9033236

R-HSA-9033236

MONDO:0008171

R-HSA-389661

R-HSA-9033241

R-HSA-71291

R-HSA-1430728

R-HSA-9609507

R-HSA-389684

R-HSA-9033233

R-HSA-9033233

R-HSA-9033235

R-HSA-9033235

R-HSA-9033236

R-HSA-9033236

MONDO:0008759

R-HSA-389661

R-HSA-71403

R-HSA-71064

R-HSA-71291

R-HSA-71406

R-HSA-1430728

R-HSA-1428517

R-HSA-6792572

R-HSA-71037

R-HSA-71401

MONDO:0008774

R-HSA-389661

R-HSA-71291

R-HSA-1430728

R-HSA-6792572

MONDO:0009401

R-HSA-70688

R-HSA-389661

R-HSA-71291

R-HSA-1430728

R-HSA-6784399

R-HSA-70679

MONDO:0009502

R-HSA-204174

R-HSA-389661