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英文名稱 AASS
中文名稱 賴氨酸酮戊二酸還原酶抗體
別    名 Alpha aminoadipic semialdehyde synthase mitochondrial; LKR/SDH; LKRSDH; LORSDH; Lysine ketoglutarate reductase; Saccharopine dehydrogenase; AASS_HUMAN.
賴氨酸酮戊二酸還原酶抗體  
說 明 書 0.2ml  
研究領(lǐng)域 腫瘤  細胞生物  信號轉(zhuǎn)導(dǎo)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
產(chǎn)品應(yīng)用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 99kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human LKRSDH (878-926aa)
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

產(chǎn)品介紹 background:
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.

Database links:

Entrez Gene: 10157 Human

Omim: 605113 Human

SwissProt: Q9UDR5 Human

Unigene: 156738 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.xy-8634R HNF3-alpha轉(zhuǎn)錄因子HNF-3α抗體
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