Lipoamide dehydrogenase is a component of the glycine cleavage system as well as an E3 component of three alpha-ketoacid dehydrogenase complexes (pyruvate-, alpha-ketoglutarate-, and branched-chain amino acid-dehydrogenase complex)
产品应用
应用
已检合格种属
预测种属
推荐稀释比例
WB
Human, Mouse, Rat
1:500-2000
IHC-P
Human, Mouse, Rat
1:50-200
IHC-F
Human, Mouse, Rat
1:50-200
IF
Human, Mouse, Rat
1:50-200
Flow-Cyt
Human
Mouse, Rat
1:50-100
ICC/IF
Human
Mouse, Rat
1:50-200
交叉反应
交叉反应: Human, Mouse, Rat
相关产品
暂无相关产品
靶标
基因名
DLD
蛋白名
Dihydrolipoyl dehydrogenase, mitochondrial
亚细胞定位
Mitochondrion matrix.
翻译后修饰
Tyrosine phosphorylated.
疾病
Note=Defects in DLD are involved in the development of congenital infantile lactic acidosis.
Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:248600]. MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation.
相似性
Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family.
功能
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.