SCP2 sterol carrier protein 2 [Homo sapiens (human)]
| Gene Symbol | SCP2 |
| Entrez Gene ID | 6342 |
| Full Name | sterol carrier protein 2 |
| Synonyms | DKFZp686C12188, DKFZp686D11188, NLTP, NSL-TP, SCP-2, SCP-CHI, SCP-X, SCPX |
| Gene Type | protein-coding |
| Organism | Homo sapiens (human) |
| Genome |
1 1p32 |
| Summary | 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. |
| Disorder | MIM: 184755 Leukoencephalopathy with dystonia and motor neuropathy, 613724 (3) |
| mRNA | NM_002979, 302344758 |
| Protein | NP_002970, 19923233 |
| Product | non-specific lipid-transfer protein isoform 1 proprotein |
| RefSeq Status | REVIEWED |
| mRNA | NM_001007098, 302318894 |
| Protein | NP_001007099, 55956775 |
| Product | non-specific lipid-transfer protein isoform 2 |
| RefSeq Status | REVIEWED |
| mRNA | NM_001007250, 302344765 |
| Protein | NP_001007251, 55956781 |
| Product | non-specific lipid-transfer protein isoform 3 proprotein |
| RefSeq Status | REVIEWED |
| mRNA | NM_001007099, 302344763 |
| Protein | NP_001007100, 55956777 |
| Product | non-specific lipid-transfer protein isoform 5 proprotein |
| RefSeq Status | REVIEWED |
| mRNA | NM_001007100, 302344764 |
| Protein | NP_001007101, 55956779 |
| Product | non-specific lipid-transfer protein isoform 4 proprotein |
| RefSeq Status | REVIEWED |
| mRNA | NM_001193599, 302344759 |
| Protein | NP_001180528, 302344760 |
| Product | non-specific lipid-transfer protein isoform 7 proprotein |
| RefSeq Status | REVIEWED |
| mRNA | NM_001193600, 302344761 |
| Protein | NP_001180529, 302344762 |
| Product | non-specific lipid-transfer protein isoform 6 proprotein |
| RefSeq Status | REVIEWED |
| mRNA | NM_001193617, 302344766 |
| Protein | NP_001180546, 302344767 |
| Product | non-specific lipid-transfer protein isoform 8 proprotein |
| RefSeq Status | REVIEWED |
| hsa00120 | Primary bile acid biosynthesis |
| hsa03320 | PPAR signaling pathway |
| hsa01100 | Metabolic pathways |
| hsa04146 | Peroxisome |
WikiPathways
| WP368 | Mitochondrial LC-Fatty Acid Beta-Oxidation |
Reactome
| REACT_17017 | Beta-oxidation of pristanoyl-CoA |
| REACT_11054 | Synthesis of bile acids and bile salts |
| REACT_22258 | Metabolism of lipids and lipoproteins |
| REACT_11040 | Bile acid and bile salt metabolism |
| REACT_11041 | Synthesis of bile acids and bile salts via 7alpha-hydroxycholesterol |
| REACT_16957 | Peroxisomal lipid metabolism |
| Homo sapiens (human) | SCP2 | NP_002970.2 |
| Pan troglodytes (chimpanzee) | SCP2 | XP_513413.2 |
| Canis lupus familiaris (dog) | SCP2 | XP_866097.1 |
| Bos taurus (cattle) | SCP2 | NP_001029162.2 |
| Mus musculus (house mouse) | Scp2 | NP_035457.1 |
| Rattus norvegicus (Norway rat) | Scp2 | NP_612517.2 |
| Gallus gallus (chicken) | SCP2 | XP_422482.2 |
| Danio rerio (zebrafish) | scp2 | NP_957159.1 |
| Drosophila melanogaster (fruit fly) | ScpX | NP_524715.2 |
| Caenorhabditis elegans | Y57A10C.6 | NP_496639.1 |
| ID | Name | Evidence |
| GO:0006694 | steroid biosynthetic process | TAS |
| GO:0006699 | bile acid biosynthetic process | TAS |
| GO:0006810 | transport | IEA |
| GO:0006869 | lipid transport | IEA |
| GO:0007031 | peroxisome organization | IEA |
| GO:0008206 | bile acid metabolic process | TAS |
| GO:0033540 | fatty acid beta-oxidation using acyl-CoA oxidase | TAS |
| GO:0044255 | cellular lipid metabolic process | EXP |
Component
| ID | Name | Evidence |
| GO:0005634 | nucleus | IDA |
| GO:0005730 | nucleolus | IDA |
| GO:0005737 | cytoplasm | IDA |
| GO:0005739 | mitochondrion | IEA |
| GO:0005777 | peroxisome | IEA |
| GO:0005782 | peroxisomal matrix | TAS |
| GO:0043231 | intracellular membrane-bounded organelle | IDA |
Function
| ID | Name | Evidence |
| GO:0005515 | protein binding | IPI |
| GO:0008289 | lipid binding | IEA |
| GO:0016740 | transferase activity | IEA |
| GO:0032934 | sterol binding | IEA |
| GO:0033814 | propanoyl-CoA C-acyltransferase activity | IEA |
| GO:0050632 | propionyl-CoA C2-trimethyltridecanoyltransferase activity | TAS |
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