- 细胞因子
- Dihydropyrimidinase and Proteins
- Regulatory proteins
- 授予称号
- Peptide Substrates
- Isoform Specific Antibodies
- Deacetylase & Demethylase Proteins
- Acetyl & Methyltransferase Proteins
- siRNA Controls
- Cell Stress and Chaperone Proteins
- 激酶
- 双加氧酶与蛋白质
- Transcription Proteins
- Carbohydrate Kinases
- 表观遗传酶抑制剂
- Growth Factors
- 脂类激酶
- Phospholipase C and Proteins
- Mutant Kinases
- Oligo Substrates
- Fluorescent and Color Proteins
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.
