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SUMF1 Rabbit pAb(bs-12366R)-50

ul/100ul/200ul
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  • ¥1180 - 2800
  • Bioss已认证
  • bs-12366R
  • 2025年10月16日
  • 产品信息以Bioss网站为准
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      50ul/100ul/200ul

    规格:50ul产品价格:¥1180.0
    规格:100ul产品价格:¥1980.0
    规格:200ul产品价格:¥2800.0
    产品编号bs-12366R
    英文名称SUMF1 Rabbit pAb
    中文名称硫酸酯酶修饰因子1抗体
    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.
    产品应用WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, ICC/IF=1:100-500, IF=1:100-500, ELISA=1:5000-10000

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    交叉反应 (Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep)
    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human SUMF1
    亚型IgG
    性状Liquid
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    理论分子量37 kDa
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    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℃. 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.
    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.

     

    应用推荐稀释比例
    {WB}{1:500-2000}
    {IHC-P}{1:100-500}
    {IHC-F}{1:100-500}
    {ICC/IF}{1:100-500}
    {IF}{1:100-500}
    {ELISA}{1:5000-10000}

     

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    SUMF1 Rabbit pAb(bs-12366R)-50ul/100ul/200ul
    ¥1180 - 2800