• 靶点：
• AASS

• 简介：
• >>Lysine degradation;>>Metabolic pathways

• 基因名称：
• AASS

• 蛋白名称：
• Alpha-aminoadipic semialdehyde synthase mitochondrial

• Human Gene Id：
• 10157

• Human Swiss Prot No：
• Q9UDR5

• Mouse Swiss Prot No：
• Q99K67

• 免疫原：
• The antiserum was produced against synthesized peptide derived from human AASS. AA range:251-300

• 特异性：
• AASS Polyclonal Antibody detects endogenous levels of AASS protein.

• 组成：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 其他名称：
• AASS;Alpha-aminoadipic semialdehyde synthase; mitochondrial;LKR/SDH

• 实测条带：
• 102kD

• 背景：
• This gene encodes a bifunctional enzyme that catalyzes the first two steps in the mammalian lysine degradation pathway. The N-terminal and the C-terminal portions of this enzyme contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in this gene are associated with familial hyperlysinemia. [provided by RefSeq, Jul 2008],

• 功能：
• catalytic activity:N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O = L-glutamate + 2-aminoadipate 6-semialdehyde + NADH.,catalytic activity:N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP(+) + H(2)O = L-lysine + 2-oxoglutarate + NADPH.,disease:Defects in AASS are the cause of hyperlysinemia [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.,function:Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.,induction:Induced by starvation.,pathway:Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 1/6.,pathway:Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lys

• 细胞定位：
• Mitochondrion .

• 组织表达：
• Expressed in all 16 tissues examined with highest expression in the liver.

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