Home
>
Product
>
Antibody
>
Rabbit Anti-SCP2/FITC Conjugated antibody
background:
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 SLCterminus. 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.
Database links:
Entrez Gene: 6342 Human
Entrez Gene: 20280 Mouse
Entrez Gene: 25541 Rat
Omim: 184755 Human
SwissProt: P22307 Human
SwissProt: P6420 Mouse
SwissProt: P11915 Rat
Unigene: 476365 Human
Unigene: 379011 Mouse
Unigene: 31887 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
固醇携带蛋白2(SCP2)参与了胆固醇的合成、代谢和转运过程.与胆囊固醇结石形成有关.
|
|