AASS Antibody Blocking Peptide(bs-11391P)-500ug

AASS Antibody Blocking Peptide

(bs-11391P)-500ug
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  • bs-11391P
  • 2025年10月16日
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      500ug

    产品编号bs-11391P
    英文名称AASS Antibody Blocking Peptide
    中文名称赖氨酸酮戊二酸还原酶封闭多肽
    英文别名Alpha aminoadipic semialdehyde synthase mitochondrial; LKR/SDH; LKRSDH; LORSDH; Lysine ketoglutarate reductase; Saccharopine dehydrogenase; AASS_HUMAN.
    纯化方法HPLC
    研究领域

    Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism

    Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers

    Signal Transduction > Metabolism > Amino Acids

    Signal Transduction > Metabolism > Mitochondrial

    亚基Homodimer
    亚细胞定位Mitochondrial
    组织特异性Expressed in all 16 tissues examined with highest expression in the liver.
    相似性In the N-terminal section; belongs to the AlaDH/PNT family.
    In the C-terminal section; belongs to the saccharopine dehydrogenase family.
    功能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.
    保存条件Shipped at 4℃. Stored 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.
    背景资料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.

     

     

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