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Rabbit Anti-SCP2  antibody (bs-2014R)  
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產(chǎn)品編號(hào) bs-2014R
英文名稱 Rabbit Anti-SCP2  antibody
中文名稱 固醇攜帶蛋白2抗體
別    名 DKFZp686C12188; DKFZp686D11188; NLTP; Nonspecific lipid transfer protein; NSL TP; OTTHUMP00000010488; Propanoyl CoA C acyltransferase; SCP 2; SCP chi; SCP X; SCP2; SCPchi; SCPX; Sterol carrier protein 2; Sterol carrier protein X; NLTP_HUMAN.  
研究領(lǐng)域 心血管  細(xì)胞生物  免疫學(xué)  染色質(zhì)和核信號(hào)  細(xì)胞周期蛋白  合成與降解  細(xì)胞分化  細(xì)胞骨架  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human,Mouse,Rat,Rabbit,Cow,Dog)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,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 59kDa
細(xì)胞定位 細(xì)胞漿 線粒體
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SCP2: 451-547/547 
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(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 This gene encodes two proteins: sterol carrier protein X(SCPx) and sterol carrier protein 2 (SCP2), as a result of transcription initiation from 2 independently regulated promoters. The transcript initiated from the proximal promoter encodes the longer SCPx protein, and the transcript initiated from the distal promoter encodes the shorter SCP2 protein, with the 2 proteins sharing a common C-terminus. Evidence suggests that the SCPx protein is a peroxisome-associated thiolase that is involved in the oxidation of branched chain fatty acids, while the SCP2 protein is thought to be an intracellular lipid transfer protein. This gene is highly expressed in organs involved in lipid metabolism, and may play a role in Zellweger syndrome, in which cells are deficient in peroxisomes and have impaired bile acid synthesis. Alternative splicing of this gene produces multiple transcript variants, some encoding different isoforms. [provided by RefSeq, Aug 2010]

Function:
Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis.

Subunit:
Interacts with PEX5.

Subcellular Location:
Cytoplasm. Mitochondrion. Note=Cytoplasmic in the liver and also associated with mitochondria especially in steroidogenic tissues.
Isoform SCPx: Peroxisome. Note=Interaction with PEX5 is essential for peroxisomal import.
Isoform SCP2: Mitochondrion (Probable).

Tissue Specificity:
Liver, fibroblasts, and placenta.

DISEASE:
Leukoencephalopathy, with dystonia and motor neuropathy (LDMN) [MIM:613724]: A syndrome characterized by leukoencephalopathy, dystonic head tremor, spasmodic torticollis and reduced tendon reflexes in lower extremities. Additional features include hyposmia, pathologic saccadic eye movements, a slight hypoacusis, accumulation of branched-chain pristanic acid in plasma, and the presence of abnormal bile alcohol glucuronides in urine. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
In the N-terminal section; belongs to the thiolase family.
Contains 1 SCP2 domain.

SWISS:
P22307

Gene ID:
6342

Database links:

Entrez Gene: 6342 Human

Entrez Gene: 20280 Mouse

Entrez Gene: 25541 Rat

Omim: 184755 Human

SwissProt: P22307 Human

SwissProt: P32020 Mouse

SwissProt: P11915 Rat

Unigene: 476365 Human

Unigene: 379011 Mouse

Unigene: 31887 Rat



固醇攜帶蛋白2(SCP2)參與了膽固醇的合成、代謝和轉(zhuǎn)運(yùn)過程.與膽囊固醇結(jié)石形成有關(guān).
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