SCP2

Protein identityUniProt · HPA

UniProt accession

P22307

Gene

SCP2

Ensembl

ENSG00000116171

Chromosome

1

Canonical length

547 aa

Protein class

Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Plasma proteins, Potential drug targets, Predicted intracellular proteins

Subcellular location

Nucleoplasm,Peroxisomes

OverviewNCBI Gene

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]

Canonical amino-acid sequenceUniProt

547 residues, UniProt reviewed canonical sequence.

>P22307|SCP2
     1  MSSSPWEPAT LRRVFVVGVG MTKFVKPGAE NSRDYPDLAE EAGKKALADA QIPYSAVDQA
    61  CVGYVFGDST CGQRAIYHSL GMTGIPIINV NNNCATGSTA LFMARQLIQG GVAECVLALG
   121  FEKMSKGSLG IKFSDRTIPT DKHVDLLINK YGLSAHPVAP QMFGYAGKEH MEKYGTKIEH
   181  FAKIGWKNHK HSVNNPYSQF QDEYSLDEVM ASKEVFDFLT ILQCCPTSDG AAAAILASEA
   241  FVQKYGLQSK AVEILAQEMM TDLPSSFEEK SIIKMVGFDM SKEAARKCYE KSGLTPNDID
   301  VIELHDCFST NELLTYEALG LCPEGQGATL VDRGDNTYGG KWVINPSGGL ISKGHPLGAT
   361  GLAQCAELCW QLRGEAGKRQ VPGAKVALQH NLGIGGAVVV TLYKMGFPEA ASSFRTHQIE
   421  AVPTSSASDG FKANLVFKEI EKKLEEEGEQ FVKKIGGIFA FKVKDGPGGK EATWVVDVKN
   481  GKGSVLPNSD KKADCTITMA DSDFLALMTG KMNPQSAFFQ GKLKITGNMG LAMKLQNLQL
   541  QPGNAKL

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against SCP2 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.24

Highest tissue expression

1,043 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about SCP2.

Disease | AllUniProt

Conditions SCP2 is implicated in, by any mechanism.

• Leukoencephalopathy with dystonia and motor neuropathy (LKDMN) MIM:613724

Disease | GeneticClinVar

27 pathogenic / likely-pathogenic of 533 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Sterol carrier protein 2 deficiency
• SCP2-related disorder
• Hepatocellular carcinoma
• Ovarian cancer

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.76

gnomAD pLI

0

gnomAD missense Z

1.26

DepMap mean gene effect

-0.1

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• alpha-linolenic acid metabolic process
• bile acid biosynthetic process
• bile acid metabolic process
• fatty acid beta-oxidation
• fatty acid beta-oxidation using acyl-CoA oxidase
• fatty acid derivative biosynthetic process
• inositol trisphosphate biosynthetic process
• intracellular cholesterol transport
• lipid hydroperoxide transport
• long-chain fatty acid biosynthetic process
• phospholipid transport
• progesterone biosynthetic process
• protein localization to plasma membrane
• regulation of lipid metabolic process
• regulation of phospholipid biosynthetic process
• steroid biosynthetic process
• unsaturated fatty acid biosynthetic process
• positive regulation of intracellular cholesterol transport
• positive regulation of steroid metabolic process

Molecular functions

• acetyl-CoA C-acyltransferase activity
• acetyl-CoA C-myristoyltransferase activity
• cholesterol binding
• cholesterol transfer activity
• fatty-acyl-CoA binding
• long-chain fatty acyl-CoA binding
• oleic acid binding
• phosphatidylcholine transfer activity
• phosphatidylinositol transfer activity
• signaling receptor binding
• propanoyl-CoA C-acyltransferase activity
• propionyl-CoA C2-trimethyltridecanoyltransferase activity

Cellular components

• cytoplasm
• cytosol
• endoplasmic reticulum
• membrane
• mitochondrion
• nucleoplasm
• peroxisomal matrix
• peroxisome
• protein-containing complex

Protein domainsUniProt · Pfam · InterPro

• SCP2 sterol-binding domain
• Thiolase-like
• Thiolase, conserved site
• Thiolase, acyl-enzyme intermediate active site
• Thiolase, N-terminal
• SCP2 sterol-binding domain superfamily
• Thiolase, N-terminal domain
• SCP-2 sterol transfer family
• Thiolase, C-terminal domain 2
• Thiolase C-terminal domain-like

KeywordsUniProt

• Acetylation
• Acyltransferase
• Alternative promoter usage
• Cytoplasm
• Endoplasmic reticulum
• Lipid metabolism
• Lipid transport
• Lipid-binding
• Mitochondrion
• Peroxisome
• Phosphoprotein
• Transferase
• Transport

InteractionsUniProt · HPA

Protein binding partners of SCP2 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• PEX5
• CAV1