SUMF1 Antibody Blocking Peptide(bs-12366P)-500ug

SUMF1 Antibody Blocking Peptid

e(bs-12366P)-500ug
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  • bs-12366P
  • 2025年10月16日
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      500ug

    产品编号bs-12366P
    英文名称SUMF1 Antibody Blocking Peptide
    中文名称硫酸酯酶修饰因子1封闭多肽
    英文别名MGC150436; AAPA3037; C alpha formylglycine generating enzyme 1; C-alpha-formylglycine-generating enzyme 1; FGE; FGly generating enzyme; MGC131853; Sulfatase modifying factor 1 [Precursor]; Sulfatase-modifying factor 1; SUMF1; SUMF1_HUMAN; UNQ3037.
    纯化方法HPLC
    亚基Monomer, homodimer and heterodimer with SUMF2.
    亚细胞定位Endoplasmic reticulum lumen.
    组织特异性Ubiquitous. Highly expressed in kidney, pancreas and liver. Detected at lower levels in leukocytes, lung, placenta, small intestine, skeletal muscle and heart.
    翻译后修饰N-glycosylated. Contains high-mannose-type oligosaccharides.
    相似性Belongs to the sulfatase-modifying factor family.
    功能Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE.
    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SUMF1 is a 374 amino acid alternatively spliced protein that localizes to the lumen of the endoplasmic reticulum and belongs to the sulfatase-modifying factor family. Expressed ubiquitously with highest expression in liver, kidney and pancreas, SUMF1 exists as either a monomer, a homodimer or a heterodimer (with SUMF2) and functions to oxidize sulfatase cysteine residues to an active FGIy residue, thereby playing an important role in sulfatase activity. Defects in the gene encoding SUMF1 are the cause of multiple sulfatase deficiency (MSD), a heterogeneous disorder characterized by metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay.

     

     

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