Reactome | BCKDH mutant tetramer [mitochondrial matrix]

Schema > DefinedSet > Entries

catalystActivities	[CatalystActivity:9865119] oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of BCKDH mutant tetramer [mitochondrial matrix]
compartment	[Compartment:5460] mitochondrial matrix
created	[InstanceEdit:9918345] Rothfels, Karen, 2024-08-14
crossReference	[DatabaseIdentifier:12020927] Mondo:0009563
dbId	9918344
disease	[Disease:9865030] maple syrup urine disease
displayName	BCKDH mutant tetramer [mitochondrial matrix]
hasMember	[Complex:R-HSA-9865099] BCKDHA:mutant BCKDHB tetramer [mitochondrial matrix] - Homo sapiens [Complex:R-HSA-9865107] mutant BCKDHA:BCKDHB tetramer [mitochondrial matrix] - Homo sapiens
inDisease	true
maxDepth	4
name	BCKDH mutant tetramer
schemaClass	DefinedSet
species	[Species:48887] Homo sapiens
stId	R-HSA-9918344

(diseaseEntity)	[EntityFunctionalStatus:9865105] loss_of_function of BCKDH mutant tetramer [mitochondrial matrix]
(physicalEntity)	[CatalystActivity:9865119] oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of BCKDH mutant tetramer [mitochondrial matrix]
(activeUnit)	[CatalystActivity:9865119] oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of BCKDH mutant tetramer [mitochondrial matrix]

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