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Details on Person Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.
| Class:Id | LiteratureReference:70020 |
|---|
| _displayName | Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. |
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| _timestamp | 2006-05-14 21:51:25 |
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| author | [Person:70021] Aevarsson, A
[Person:70022] Chuang, JL
[Person:70023] Wynn, RM
[Person:70024] Turley, S
[Person:70025] Chuang, DT
[Person:70026] Hol, WG |
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| journal | Structure Fold Des |
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| modified | [InstanceEdit:180006] D'Eustachio, P, 2006-05-14 21:48:50
[InstanceEdit:180006] D'Eustachio, P, 2006-05-14 21:48:50
[InstanceEdit:180007] D'Eustachio, P, 2006-05-14 21:51:25 |
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| pages | 277-91 |
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| pubMedIdentifier | 10745006 |
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| title | Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. |
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| volume | 8 |
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| year | 2000 |
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| (literatureReference) | [Pathway:9859138] BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV [Homo sapiens]
[Reaction:9859148] BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV [Homo sapiens]
[Pathway:9865114] Maple Syrup Urine Disease [Homo sapiens]
[FailedReaction:9865121] BCKDHA or BCKDHB loss-of-function mutants don't synthesize BCAA-CoA [Homo sapiens]
[Pathway:9865125] Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD [Homo sapiens]
[Complex:70019] BCKDH [mitochondrial matrix] [Homo sapiens]
[Complex:5693120] p-BCKDH [mitochondrial matrix] [Homo sapiens]
[Complex:6792583] BCKDH [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:9865040] G290R BCKDHA [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:9865047] H206R BCKDHB [mitochondrial matrix] [Homo sapiens] |
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No pathways have been reviewed or authored by Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. (70020)
