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AL4A1 mouse mAb

BYmab-07989

  • 20UL ¥460 50UL ¥945 100UL ¥1350
  • 货期: 3个工作日

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宿主
反应性
应用
WB
分子量(DA)
60kD
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
来源
Monoclonal, Mouse,IgG
组成(Formulation)
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.103% sodium azide.
稀释比例
WB 1:500-2000
纯化工艺(Immunogen)
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
浓度
1 mg/ml
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
蛋白名称
AL4A1
简称
AL4A1
其他名称
Delta-1-pyrroline-5-carboxylate dehydrogenase, 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
人基因ID
人蛋白质序列数据库
小鼠基因ID
小鼠蛋白质序列数据库
大鼠基因ID
大鼠蛋白质序列数据库
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.
储存(Storage)
-20°C/1 year

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AL4A1 mouse mAb

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