Sterol Carrier Protein 2 (SCP2) Reagents

Sterol carrier protein 2

Plays a crucial role in the peroxisomal oxidation of branched-chain fatty acids. Catalyzes the last step of the peroxisomal beta-oxidation of branched chain fatty acids and the side chain of the bile acid intermediates di- and trihydroxycoprostanic acids (DHCA and THCA). Also active with medium and long straight chain 3-oxoacyl-CoAs. Stimulates the microsomal conversion of 7-dehydrocholesterol to cholesterol and transfers phosphatidylcholine and 7-dehydrocholesterol between membrances, in vitro. Isoforms SCP2 and SCPx cooperate in peroxisomal oxidation of certain naturally occurring tetramethyl-branched fatty acyl-CoAs.; Mediates the transfer of all common phospholipids, cholesterol and gangliosides from the endoplasmic reticulum to the plasma membrane. May play a role in regulating steroidogenesis. Stimulates the microsomal conversion of 7-dehydrocholesterol to cholesterol. Also binds fatty acids and fatty acyl Coenzyme A (CoA) such as phytanoyl-CoA. Involved in the regulation phospholipid synthesis in endoplasmic reticulum enhancing the incorporation of exogenous fatty acid into glycerides. Seems to stimulate the rate-limiting step in phosphatidic acid formation mediated by GPAT3. Isoforms SCP2 and SCPx cooperate in peroxisomal oxidation of certain naturally occurring tetramethyl-branched fatty acyl-CoAs.

Leukoencephalopathy with dystonia and motor neuropathy (LKDMN)

[Isoform SCP2]: Peroxisome. Cytoplasm. Mitochondrion. Endoplasmic reticulum. Mitochondrion.; [Isoform SCPx]: Peroxisome.

Thiolase family

Liver, fibroblasts, and placenta.

Transport

DKFZp686C12188; DKFZp686D11188; NLTP; NLTP_HUMAN; Non-specific lipid-transfer protein; Nonspecific lipid transfer protein; NSL TP; NSL-TP; OTTHUMP00000010488; OTTHUMP00000231766; OTTHUMP00000231767; OTTHUMP00000231768; OTTHUMP00000231769; OTTHUMP00000231770; OTTHUMP00000231772; OTTHUMP00000231773; OTTHUMP00000231774; OTTHUMP00000231776; OTTHUMP00000234662; Propanoyl CoA C acyltransferase; Propanoyl-CoA C-acyltransferase; SCP 2; SCP chi ; SCP X; SCP-2; SCP-chi; SCP-X; SCP2; SCPchi ; SCPX; Sterol carrier protein 2; Sterol carrier protein X

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.