DAR - Bologna Biocomputing Group

Search results (Reactome ID: R-HSA-70895)

DiseaseID	Gene	UniProt	EC numbers	Reactomes	Expanded Reactomes	Reactome roots	Reactions
MONDO:0008760	ACAT1	P24752	2.3.1.9	R-HSA-77111 R-HSA-77111 Reactome Name: Synthesis of Ketone Bodies See in Reactome R-HSA-77108 R-HSA-77108 Reactome Name: Utilization of Ketone Bodies See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome	R-HSA-556833 R-HSA-556833 Reactome Name: Metabolism of lipids See in Reactome R-HSA-74182 R-HSA-74182 Reactome Name: Ketone body metabolism See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome	Metabolism	R-HSA-70844 ( R-HSA-70895 ) R-HSA-70844 Reaction Name: alpha-methyl-acetoacetyl-CoA + CoA => propionyl-CoA + acetyl-CoA See in Reactome R-HSA-73916 ( R-HSA-77111 ) R-HSA-73916 Reaction Name: 2 acetyl-CoA <=> acetoacetyl-CoA + CoA See in Reactome R-HSA-74181 ( R-HSA-77108 ) R-HSA-74181 Reaction Name: acetoacetyl-CoA + CoA <=> 2 acetyl-CoA See in Reactome
MONDO:0008861	MCCC1	Q96RQ3	6.4.1.4	R-HSA-196780 R-HSA-196780 Reactome Name: Biotin transport and metabolism 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-196849 R-HSA-196849 Reactome Name: Metabolism of water-soluble vitamins and cofactors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-196854 R-HSA-196854 Reactome Name: Metabolism of vitamins and cofactors See in Reactome	Metabolism	R-HSA-2993799 ( R-HSA-196780 ) R-HSA-2993799 Reaction Name: HLCS biotinylates 6xMCCC1:6xMCCC2 See in Reactome R-HSA-3065959 ( R-HSA-196780 ) R-HSA-3065959 Reaction Name: An unknown protease degrades hCBXs See in Reactome R-HSA-3323111 ( R-HSA-196780 ) R-HSA-3323111 Reaction Name: Cytoslic carboxylases translocate to mitochondrial matrix See in Reactome R-HSA-508308 ( R-HSA-70895 ) R-HSA-508308 Reaction Name: beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O <=> beta-methylcrotonyl-CoA + ATP + CO2 [MCCA] See in Reactome R-HSA-70773 ( R-HSA-70895 ) R-HSA-70773 Reaction Name: beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] See in Reactome
MONDO:0008862	MCCC2	Q9HCC0	6.4.1.4	R-HSA-196780 R-HSA-196780 Reactome Name: Biotin transport and metabolism 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-196849 R-HSA-196849 Reactome Name: Metabolism of water-soluble vitamins and cofactors See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-196854 R-HSA-196854 Reactome Name: Metabolism of vitamins and cofactors See in Reactome	Metabolism	R-HSA-2993799 ( R-HSA-196780 ) R-HSA-2993799 Reaction Name: HLCS biotinylates 6xMCCC1:6xMCCC2 See in Reactome R-HSA-3065959 ( R-HSA-196780 ) R-HSA-3065959 Reaction Name: An unknown protease degrades hCBXs See in Reactome R-HSA-3323111 ( R-HSA-196780 ) R-HSA-3323111 Reaction Name: Cytoslic carboxylases translocate to mitochondrial matrix See in Reactome R-HSA-508308 ( R-HSA-70895 ) R-HSA-508308 Reaction Name: beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O <=> beta-methylcrotonyl-CoA + ATP + CO2 [MCCA] See in Reactome R-HSA-70773 ( R-HSA-70895 ) R-HSA-70773 Reaction Name: beta-methylcrotonyl-CoA + ATP + CO2 <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O [MCCA] See in Reactome
MONDO:0009475	IVD	P26440	1.3.8.1 1.3.8.4	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-70745 ( R-HSA-70895 ) R-HSA-70745 Reaction Name: isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 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:0009603	HIBCH	Q6NVY1	3.1.2.4	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-70881 ( R-HSA-70895 ) R-HSA-70881 Reaction Name: beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA See in Reactome
MONDO:0009610	AUH	Q13825	4.2.1.18 4.2.1.56	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-70785 ( R-HSA-70895 ) R-HSA-70785 Reaction Name: beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA See in Reactome
MONDO:0012392	ACADSB	P45954	1.3.8.5	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-70800 ( R-HSA-70895 ) R-HSA-70800 Reaction Name: alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2 See in Reactome
MONDO:0012648	ACAD8	Q9UKU7	1.3.8.-	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-70859 ( R-HSA-70895 ) R-HSA-70859 Reaction Name: isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2 See in Reactome
MONDO:0013579	ALDH6A1	Q02252	1.2.1.27 1.2.1.18	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-70893 ( R-HSA-70895 ) R-HSA-70893 Reaction Name: methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ See in Reactome
MONDO:0013970	BCKDK	O14874	2.7.11.4	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
MONDO:0014057	PPM1K	Q8N3J5	3.1.3.16	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-5693153 ( R-HSA-70895 ) R-HSA-5693153 Reaction Name: PPM1K dephosphorylates p-BCKDH See in Reactome
MONDO:0014563	ECHS1	P30084	5.3.3.8 4.2.1.17	R-HSA-77346 R-HSA-77346 Reactome Name: Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA See in Reactome R-HSA-77352 R-HSA-77352 Reactome Name: Beta oxidation of butanoyl-CoA to acetyl-CoA See in Reactome R-HSA-77350 R-HSA-77350 Reactome Name: Beta oxidation of hexanoyl-CoA to butanoyl-CoA See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-77310 R-HSA-77310 Reactome Name: Beta oxidation of lauroyl-CoA to decanoyl-CoA-CoA See in Reactome R-HSA-77348 R-HSA-77348 Reactome Name: Beta oxidation of octanoyl-CoA to hexanoyl-CoA See in Reactome	R-HSA-77289 R-HSA-77289 Reactome Name: Mitochondrial Fatty Acid Beta-Oxidation See in Reactome R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-77286 R-HSA-77286 Reactome Name: mitochondrial fatty acid beta-oxidation of saturated fatty acids See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-8978868 R-HSA-8978868 Reactome Name: Fatty acid metabolism See in Reactome R-HSA-556833 R-HSA-556833 Reactome Name: Metabolism of lipids See in Reactome	Metabolism	R-HSA-70870 ( R-HSA-70895 ) R-HSA-70870 Reaction Name: ECHS1 hydrates methacrylyl-CoA See in Reactome R-HSA-77256 ( R-HSA-77310 ) R-HSA-77256 Reaction Name: 2-trans-Dodecenoyl-CoA+H2O<=>(S)-3-Hydroxydodecanoyl-CoA See in Reactome R-HSA-77314 ( R-HSA-77352 ) R-HSA-77314 Reaction Name: Crotonoyl-CoA+H2O<=>(S)-3-Hydroxybutanoyl-CoA See in Reactome R-HSA-77325 ( R-HSA-77350 ) R-HSA-77325 Reaction Name: trans-Hex-2-enoyl-CoA+H2O<=>(S)-Hydroxyhexanoyl-CoA See in Reactome R-HSA-77333 ( R-HSA-77348 ) R-HSA-77333 Reaction Name: trans-Oct-2-enoyl-CoA+H2O<=>(S)-Hydroxyoctanoyl-CoA See in Reactome R-HSA-77344 ( R-HSA-77346 ) R-HSA-77344 Reaction Name: trans-Dec-2-enoyl-CoA+H2O<=>(S)-Hydroxydecanoyl-CoA 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:0018322	HSD17B10	Q99714	1.1.1.62 1.1.1.35 1.1.1.239 1.1.1.178 1.1.1.159 1.1.1.53	R-HSA-6785470 R-HSA-6785470 Reactome Name: tRNA processing in the mitochondrion See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-6787450 R-HSA-6787450 Reactome Name: tRNA modification in the mitochondrion See in Reactome R-HSA-8868766 R-HSA-8868766 Reactome Name: rRNA processing in the mitochondrion See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-8953854 R-HSA-8953854 Reactome Name: Metabolism of RNA See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-72312 R-HSA-72312 Reactome Name: rRNA processing See in Reactome R-HSA-72306 R-HSA-72306 Reactome Name: tRNA processing See in Reactome	Metabolism of RNA Metabolism	R-HSA-508369 ( R-HSA-70895 ) R-HSA-508369 Reaction Name: alpha-methylacetoacetyl-CoA + NADH + H+ <=> alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ See in Reactome R-HSA-6785722 ( R-HSA-6785470; R-HSA-8868766 ) R-HSA-6785722 Reaction Name: Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the H strand transcript See in Reactome R-HSA-6786854 ( R-HSA-6785470 ) R-HSA-6786854 Reaction Name: Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the L strand transcript See in Reactome R-HSA-6787591 ( R-HSA-6787450 ) R-HSA-6787591 Reaction Name: TRMT10C:HSD17B10 (TRMT10C:SDR5C1) of mitochondrial RNase P methylates guanosine-9 in tRNA yielding 1-methylguanosine-9 See in Reactome R-HSA-6787594 ( R-HSA-6787450 ) R-HSA-6787594 Reaction Name: TRMT10C:HSD17B10 (TRMT10C:SDR5C1) methylates adenosine-9 in tRNA yielding 1-methyladenosine-9 See in Reactome R-HSA-70837 ( R-HSA-70895 ) R-HSA-70837 Reaction Name: alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ <=> alpha-methylacetoacetyl-CoA + NADH + H+ See in Reactome
MONDO:0018323	HSD17B10	Q99714	1.1.1.62 1.1.1.35 1.1.1.239 1.1.1.178 1.1.1.159 1.1.1.53	R-HSA-6785470 R-HSA-6785470 Reactome Name: tRNA processing in the mitochondrion See in Reactome R-HSA-70895 R-HSA-70895 Reactome Name: Branched-chain amino acid catabolism See in Reactome R-HSA-6787450 R-HSA-6787450 Reactome Name: tRNA modification in the mitochondrion See in Reactome R-HSA-8868766 R-HSA-8868766 Reactome Name: rRNA processing in the mitochondrion See in Reactome	R-HSA-71291 R-HSA-71291 Reactome Name: Metabolism of amino acids and derivatives See in Reactome R-HSA-8953854 R-HSA-8953854 Reactome Name: Metabolism of RNA See in Reactome R-HSA-1430728 R-HSA-1430728 Reactome Name: Metabolism See in Reactome R-HSA-72312 R-HSA-72312 Reactome Name: rRNA processing See in Reactome R-HSA-72306 R-HSA-72306 Reactome Name: tRNA processing See in Reactome	Metabolism of RNA Metabolism	R-HSA-508369 ( R-HSA-70895 ) R-HSA-508369 Reaction Name: alpha-methylacetoacetyl-CoA + NADH + H+ <=> alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ See in Reactome R-HSA-6785722 ( R-HSA-6785470; R-HSA-8868766 ) R-HSA-6785722 Reaction Name: Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the H strand transcript See in Reactome R-HSA-6786854 ( R-HSA-6785470 ) R-HSA-6786854 Reaction Name: Mitochondrial RNase P (mtRNase P) cleaves the 5' ends of pre-tRNAs and ELAC2 (RNase Z) cleaves the 3' ends of pre-tRNAs in the L strand transcript See in Reactome R-HSA-6787591 ( R-HSA-6787450 ) R-HSA-6787591 Reaction Name: TRMT10C:HSD17B10 (TRMT10C:SDR5C1) of mitochondrial RNase P methylates guanosine-9 in tRNA yielding 1-methylguanosine-9 See in Reactome R-HSA-6787594 ( R-HSA-6787450 ) R-HSA-6787594 Reaction Name: TRMT10C:HSD17B10 (TRMT10C:SDR5C1) methylates adenosine-9 in tRNA yielding 1-methyladenosine-9 See in Reactome R-HSA-70837 ( R-HSA-70895 ) R-HSA-70837 Reaction Name: alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ <=> alpha-methylacetoacetyl-CoA + 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:0100058	BCAT2	O15382	2.6.1.42	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-508179 ( R-HSA-70895 ) R-HSA-508179 Reaction Name: a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] See in Reactome R-HSA-70724 ( R-HSA-70895 ) R-HSA-70724 Reaction Name: leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] See in Reactome

Number of enzymes:	18
Number of disease:	23
Number of reactomes:	20
Number of reactions:	39

Search summary (Reactome ID: R-HSA-70895)

Search results (Reactome ID: R-HSA-70895)

MONDO:0008760

R-HSA-77111

R-HSA-77108

R-HSA-70895

R-HSA-556833

R-HSA-74182

R-HSA-1430728

R-HSA-71291

R-HSA-70844

R-HSA-73916

R-HSA-74181

MONDO:0008861

R-HSA-196780

R-HSA-70895

R-HSA-71291

R-HSA-196849

R-HSA-1430728

R-HSA-196854

R-HSA-2993799

R-HSA-3065959

R-HSA-3323111

R-HSA-508308

R-HSA-70773

MONDO:0008862

R-HSA-196780

R-HSA-70895

R-HSA-71291

R-HSA-196849

R-HSA-1430728

R-HSA-196854

R-HSA-2993799

R-HSA-3065959

R-HSA-3323111

R-HSA-508308

R-HSA-70773

MONDO:0009475

R-HSA-70895

R-HSA-71291

R-HSA-1430728

R-HSA-70745

MONDO:0009529

R-HSA-71403

R-HSA-204174

R-HSA-70895

R-HSA-70268

R-HSA-71064

R-HSA-6783984

R-HSA-389661

R-HSA-5362517

R-HSA-71406

R-HSA-71291

R-HSA-9006931

R-HSA-1430728

R-HSA-1428517

R-HSA-162582

R-HSA-203946

R-HSA-204169

R-HSA-5693148

R-HSA-5693153

R-HSA-5694018

R-HSA-6792572

R-HSA-70713

R-HSA-71037

R-HSA-71397

R-HSA-71401

MONDO:0009603

R-HSA-70895

R-HSA-71291

R-HSA-1430728

R-HSA-70881

MONDO:0009610

R-HSA-70895

R-HSA-71291

R-HSA-1430728

R-HSA-70785

MONDO:0012392

R-HSA-70895

R-HSA-71291