免疫原
Synthesized peptide derived from human AL4A1 AA range: 39-89
特异性
This antibody detects endogenous levels of AL4A1 at Human/Mouse/Rat
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.103% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（Background）
This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2009],
功能
catalytic activity:(S)-1-pyrroline-5-carboxylate + NAD(P)(+) + 2 H(2)O = L-glutamate + NAD(P)H.,disease:Defects in ALDH4A1 are the cause of hyperprolinemia type II (HPII) [MIM:239510]. HPII is characterized by the accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. The disorder may be causally related to neurologic manifestations, including seizures and mental retardation.,function:Irreversible conversion of delta-1-pyrroline-5-carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes.,pathway:Amino-acid degradation; L-proline degradation into L-glutamate; L-glutamate from L-proline: step 2/2.,similarity:Belongs to the aldehyde dehydrogena
基因名称（Gene Name）
ALDH4A1 ALDH4 P5CDH
其他名称
AL4A1_HUMAN;aldehyde dehydrogenase 4;aldehyde dehydrogenase 4 family;member A1;Aldehyde dehydrogenase family 4 member A1;Aldehyde dehydrogenase;family 4;subfamily A;member 1;ALDH4;aldh4a1;Delta 1 pyrroline 5 carboxylate dehydrogenase;mitochondrial;Delta-1-pyrroline-5-carboxylate dehydrogenase;L-glutamate gamma-semialdehyde dehydrogenase;mitochondrial delta-1-pyrroline 5-carboxylate dehydrogenase;P5C dehydrogenase;P5CD;P5CDh;P5CDhL;P5CDhS;Pyrroline-5-carboxylate dehydrogenase;RP11 128M1.1;AL4A1;ALDH4A1 ALDH4 P5CDH;mitochondrial (P5C dehydrogenase) (EC 1.5.1.12) (Aldehyde dehydrogenase family 4 member A1)
Fields
>>Alanine, aspartate and glutamate metabolism;>>Arginine and proline metabolism;>>Metabolic pathways
细胞定位
Mitochondrion matrix.
组织表达
Highest expression is found in liver followed by skeletal muscle, kidney, heart, brain, placenta, lung and pancreas.
联系我们
关注我们
您当前的位置： 
说明书
成功添加到购物车
