硫酸酯酶修饰因子1抗体
规格：1mg/1ml
英文名： SUMF1
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-m
分子量： 37kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human SUMF1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆
硫酸酯酶修饰因子1抗体产品介绍：background: 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硫酸酯酶修饰因子1抗体, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Function: 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. Subunit: Monomer, homodimer and heterodimer with SUMF2. Subcellular Location: Endoplasmic reticulum lumen. Tissue Specificity: Ubiquitous. Highly expressed in kidney, pancreas and liver. Detected at lower levels in leukocytes, lung, placenta, small intestine, skeletal muscle and heart. Post-translational modifications: N-glycosylated. Contains high-mannose-type oligosaccharides. 硫酸酯酶修饰因子1抗体DISEASE: Defects in SUMF1 are the cause of multiple sulfatase deficiency (MSD) [MIM:272200]. MSD is a clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Inheritance is autosomal recessive. Similarity: Belongs to the sulfatase-modifying factor family. Database links: UniProtKB/Swiss-Prot: Q8NBK3.3 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
硫酸酯酶修饰因子1抗体产品应用：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.
研究领域：细胞生物 发育生物学 神经生物学 信号转导 干细胞
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid