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C-4 Methylsterol Oxidase/MSMO1 Rabbit pAb (bs-2947R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢價
產品編號 bs-2947R
英文名稱 C-4 Methylsterol Oxidase/MSMO1 Rabbit pAb
中文名稱 甲基固醇羥化酶單加氧酶1抗體
別    名 1500001G16Rik; C 4 methyl sterol; C78600; DESP4; ERG25; Methylsterol monooxygenase 1; MGC104344; MSMO1; MSMO1_HUMAN; OTTHUMP00000219757; OTTHUMP00000219758; OTTHUMP00000219759; OTTHUMP00000219760; sterol C4 methyl oxidase like.  
研究領域 腫瘤  心血管  細胞生物  信號轉導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Cow,Dog,Horse)
產品應用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 35 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MSMO1: 131-230/293 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 Belonging to the sterol desaturase family, ERG25 is a 293 amino acid enzyme that performs the first of three enzymatic steps required to remove the two methyl groups leading to cholesterol. ERG25 is a multi-pass membrane protein that resides within the plasma membrane and the membrane of the endoplasmic reticulum. Concentrations of ERG25 mRNA increase when subjected to low iron growth conditions, suggesting that the enzyme is not regulated by iron but by an end product of the ergosterol pathway. Low-density lipoprotein (LDL) downregulates ERG25 expression in the vascular wall, an event that may be inhibited via SREBP overexpression. ERG25 is encoded by a gene located on human chromosome 4q32.3.

Function:
Catalyzes the first step in the removal of the two C-4 methyl groups of 4,4-dimethylzymosterol.

Subcellular Location:
Endoplasmic reticulum membrane; Multi-pass membrane protein.

DISEASE:
Microcephaly, congenital cataract, and psoriasiform dermatitis (MCCPD) [MIM:616834]: An autosomal recessive inborn error of cholesterol metabolism characterized by accumulation of a large amount of methylsterols, particularly dimethylsterols, in affected individuals. Patients manifest psoriasiform dermatitis, arthralgias, congenital cataracts, microcephaly, and developmental delay. {ECO:0000269|PubMed:21285510, ECO:0000269|PubMed:24144731}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the sterol desaturase family.

SWISS:
Q15800

Gene ID:
6307

Database links:

Entrez Gene: 6307 Human

Entrez Gene: 66234 Mouse

Entrez Gene: 140910 Rat

Omim: 607545 Human

SwissProt: Q15800 Human

SwissProt: Q9CRA4 Mouse

SwissProt: O35532 Rat

Unigene: 105269 Human

Unigene: 30119 Mouse

Unigene: 7167 Rat



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