- Peptide Substrates
- Binding Proteins
- Secondary Antibodies
- Regulatory proteins
- 脂类激酶
- 双加氧酶与蛋白质
- 脂质底物
- E2
- Assay Buffer and Co-factors
- Methyltransferases
- Acetyltransferases
- Transcription Proteins
- COVID-19 ELISA Kits
- Tau Proteins
- Microtubule & Actin Associated Proteins
- Carbohydrate Substrates
- COVID-19 Proteins
- Chemokines
- 标记抗体
- 授予称号
- E3
Overview:
PDHA1pyruvatedehydrogenase(lipoamide)alpha1isamemberofthepyruvatedehydrogenase(PDH)complex.PDHA1isanuclear-encodedmitochondrialmultienzymecomplexwhichcatalyzestheoverallconversionofpyruvatetoacetyl-CoAandCO(2),andprovidestheprimarylinkbetweenglycolysisandthetricarboxylicacid(TCA)cycle.PDHA1playsacriticalroleinthebrainthatusuallyobtainsallofitsenergyfromtheaerobicoxidationofglucose(1).MutationsinthePDHA1areassociatedwithpyruvatedehydrogenaseE1-alphadeficiencyandX-linkedLeighsyndrome(2).
GeneAliases:
PDHA;PDHCE1A;PHE1A
GenbankNumber:
NM_000284
References:
1.Brown,G.K.et.al:Pyruvatedehydrogenasedeficiency.J.Med.Genet.31:875-879,1994.2.Chun,K.et.al:MutationsintheX-linkedE1-alphasubunitofpyruvatedehydrogenaseleADIngtodeficiencyofthepyruvatedehydrogenasecomplex.Hum.Molec.Genet.2:449-454,1993.
