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RabbitAnti-AASS Rabbit pAb  antibody (bs-11391R)
~~~促销,代码KXJ230206~~~
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价

产品编号 bs-11391R
英文名称 AASS Rabbit pAb
中文名称 赖氨酸酮戊二酸还原酶抗体
别    名 LKR/SDH; LKRSDH; LORSDH; AASS_HUMAN; AASS;   
研究领域 肿瘤  细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 99 kDa
检测分子量 95
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LKRSDH: 878-926/926 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 Alpha-aminoadipic semialdehyde synthase (AASS), also designated lysine ketoglutarate reductase (LKR) or saccharopine dehydrogenase (SDH), is a 926 amino acid protein that exists as a homodimer in the mitochondria. AASS acts as a bifunctional enzyme containing the lysine alpha-ketoglutarate reductase (LKR) and saccharopine dehydrogenase activities that catalyzes the first two steps in lysine degradation. It is widely expressed with highest expression in liver and transcription of the AASS gene is induced upon starvation. Mutations in the gene encoding AASS result in various forms familial hyperlysinemias (FH), autosomal recessive disorders characterized by hyperlysinemia, lysinuria, and variable saccharopinuria. However, no adverse mental or physical effects have been found in patients with hyperlysinemia.

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.

Subunit:
Homodimer

Subcellular Location:
Mitochondrial

Tissue Specificity:
Expressed in all 16 tissues examined with highest expression in the liver.

DISEASE:
Defects in AASS are the cause of hyperlysinemia (HYPLYS) [MIM:238700]. Hyperlysinemia is an autosomal recessive condition characterized by hyperlysinemia lysinuria and variable saccharopinuria.

Similarity:
In the N-terminal section; belongs to the AlaDH/PNT family.
In the C-terminal section; belongs to the saccharopine dehydrogenase family.

SWISS:
Q9UDR5

Gene ID:
10157

Database links:

Entrez Gene: 10157 Human

Omim: 605113 Human

SwissProt: Q9UDR5 Human

Unigene: 156738 Human



产品图片
Sample: Liver(Mouse) Lysate at 40 ug Kidney (Mouse) Lysate at 40 ug Primary: Anti- AASS (bs-11391R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 99 kD Observed band size: 95 kD
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