免疫原
Synthesized peptide derived from human NLTP AA range: 338-388
特异性
This antibody detects endogenous levels of NLTP at Human/Mouse/Rat
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（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 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],
功能
catalytic activity:3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholanoyl-CoA + propanoyl-CoA = CoA + 3-alpha,7-alpha,12-alpha-trihydroxy-24-oxo-5-beta-cholestanoyl-CoA.,caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:SCP2 is present in low levels in subjects with Zellweger syndrome (cerebro-hepatic-renal syndrome), whose cells are deficient in peroxisomes and who have an associated impairment in plasmalogen and bile acid synthesis and catabolism of phytanic acid and very-long-chain fatty acids.,function:Mediates in vitro the transfer of all common phospholipids, cholesterol and gangliosides between membranes. May play a role in regulating steroidogenesis.,similarity:Belongs to the thiolase family.,similarity:Contains 1 SCP2 domain.,similarity:In the N-terminal section; belongs to the thiolase fami
Fields
>>Primary bile acid biosynthesis;>>Biosynthesis of unsaturated fatty acids;>>Metabolic pathways;>>Fatty acid metabolism;>>PPAR signaling pathway;>>Peroxisome
细胞定位
[Isoform SCP2]: Peroxisome . Cytoplasm . Mitochondrion . Endoplasmic reticulum . Mitochondrion .; [Isoform SCPx]: Peroxisome .
组织表达
Liver, fibroblasts, and placenta.
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