Gene/Proteome Database (LMPD)
LMPD ID
LMP002045
Gene ID
Species
Homo sapiens (Human)
Gene Name
steroidogenic acute regulatory protein
Gene Symbol
Synonyms
STARD1
Alternate Names
steroidogenic acute regulatory protein, mitochondrial; cholesterol trafficker; START domain containing 1; steroidogenic acute regulator; steroid acute regulatory protein; START domain-containing protein 1; mitochondrial steroid acute regulatory protein; StAR-related lipid transfer (START) domain containing 1
Chromosome
8
Map Location
8p11.2
Summary
The protein encoded by this gene plays a key role in the acute regulation of steroid hormone synthesis by enhancing the conversion of cholesterol into pregnenolone. This protein permits the cleavage of cholesterol into pregnenolone by mediating the transport of cholesterol from the outer mitochondrial membrane to the inner mitochondrial membrane. Mutations in this gene are a cause of congenital lipoid adrenal hyperplasia (CLAH), also called lipoid CAH. A pseudogene of this gene is located on chromosome 13. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
steroidogenic acute regulatory protein, mitochondrial | |
---|---|
Refseq ID | NP_000340 |
Protein GI | 56243551 |
UniProt ID | P49675 |
mRNA ID | NM_000349 |
Length | 285 |
RefSeq Status | REVIEWED |
MLLATFKLCAGSSYRHMRNMKGLRQQAVMAISQELNRRALGGPTPSTWINQVRRRSSLLGSRLEETLYSDQELAYLQQGEEAMQKALGILSNQEGWKKESQQDNGDKVMSKVVPDVGKVFRLEVVVDQPMERLYEELVERMEAMGEWNPNVKEIKVLQKIGKDTFITHELAAEAAGNLVGPRDFVSVRCAKRRGSTCVLAGMATDFGNMPEQKGVIRAEHGPTCMVLHPLAGSPSKTKLTWLLSIDLKGWLPKSIINQVLSQTQVDFANHLRKRLESHPASEARC |
Gene Information
Entrez Gene ID
Gene Name
steroidogenic acute regulatory protein
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
GO ID | Source | Type | Description |
---|---|---|---|
GO:0005829 | IEA:Ensembl | C | cytosol |
GO:0030061 | IEA:Ensembl | C | mitochondrial crista |
GO:0005758 | TAS:Reactome | C | mitochondrial intermembrane space |
GO:0043005 | IEA:Ensembl | C | neuron projection |
GO:0043025 | IEA:Ensembl | C | neuronal cell body |
GO:0015485 | IEA:Ensembl | F | cholesterol binding |
GO:0006700 | TAS:Reactome | P | C21-steroid hormone biosynthetic process |
GO:0006699 | IEA:Ensembl | P | bile acid biosynthetic process |
GO:0018879 | IEA:Ensembl | P | biphenyl metabolic process |
GO:0007420 | IEA:Ensembl | P | brain development |
GO:0071312 | IEA:Ensembl | P | cellular response to alkaloid |
GO:0071236 | IEA:Ensembl | P | cellular response to antibiotic |
GO:0071320 | IEA:Ensembl | P | cellular response to cAMP |
GO:0071276 | IEA:Ensembl | P | cellular response to cadmium ion |
GO:0071549 | IEA:Ensembl | P | cellular response to dexamethasone stimulus |
GO:0071872 | IEA:Ensembl | P | cellular response to epinephrine stimulus |
GO:0044344 | IEA:Ensembl | P | cellular response to fibroblast growth factor stimulus |
GO:0071372 | IEA:Ensembl | P | cellular response to follicle-stimulating hormone stimulus |
GO:0071333 | IEA:Ensembl | P | cellular response to glucose stimulus |
GO:0071378 | IEA:Ensembl | P | cellular response to growth hormone stimulus |
GO:0032869 | IEA:Ensembl | P | cellular response to insulin stimulus |
GO:0035457 | IEA:Ensembl | P | cellular response to interferon-alpha |
GO:0071346 | IEA:Ensembl | P | cellular response to interferon-gamma |
GO:0071222 | IEA:Ensembl | P | cellular response to lipopolysaccharide |
GO:0071373 | IEA:Ensembl | P | cellular response to luteinizing hormone stimulus |
GO:0071560 | IEA:Ensembl | P | cellular response to transforming growth factor beta stimulus |
GO:0008203 | IEA:UniProtKB-UniPathway | P | cholesterol metabolic process |
GO:0042747 | IEA:Ensembl | P | circadian sleep/wake cycle, REM sleep |
GO:0018894 | IEA:Ensembl | P | dibenzo-p-dioxin metabolic process |
GO:0016101 | IEA:Ensembl | P | diterpenoid metabolic process |
GO:0006703 | IEA:Ensembl | P | estrogen biosynthetic process |
GO:0050756 | IEA:Ensembl | P | fractalkine metabolic process |
GO:0008211 | IEA:Ensembl | P | glucocorticoid metabolic process |
GO:0017143 | IEA:Ensembl | P | insecticide metabolic process |
GO:0032367 | IEA:Ensembl | P | intracellular cholesterol transport |
GO:0008584 | IEA:Ensembl | P | male gonad development |
GO:0043524 | IEA:Ensembl | P | negative regulation of neuron apoptotic process |
GO:0018958 | IEA:Ensembl | P | phenol-containing compound metabolic process |
GO:0018963 | IEA:Ensembl | P | phthalate metabolic process |
GO:0010628 | IEA:Ensembl | P | positive regulation of gene expression |
GO:0050769 | IEA:Ensembl | P | positive regulation of neurogenesis |
GO:0006701 | IEA:Ensembl | P | progesterone biosynthetic process |
GO:0048168 | IEA:Ensembl | P | regulation of neuronal synaptic plasticity |
GO:0050810 | IEA:Ensembl | P | regulation of steroid biosynthetic process |
GO:0014823 | IEA:Ensembl | P | response to activity |
GO:0051412 | IEA:Ensembl | P | response to corticosterone |
GO:0042493 | IEA:Ensembl | P | response to drug |
GO:0043627 | IEA:Ensembl | P | response to estrogen |
GO:0045471 | IEA:Ensembl | P | response to ethanol |
GO:0060992 | IEA:Ensembl | P | response to fungicide |
GO:0009635 | IEA:Ensembl | P | response to herbicide |
GO:0042542 | IEA:Ensembl | P | response to hydrogen peroxide |
GO:0010212 | IEA:Ensembl | P | response to ionizing radiation |
GO:0010288 | IEA:Ensembl | P | response to lead ion |
GO:0044321 | IEA:Ensembl | P | response to leptin |
GO:0035094 | IEA:Ensembl | P | response to nicotine |
GO:0007584 | IEA:Ensembl | P | response to nutrient |
GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
GO:0006694 | TAS:ProtInc | P | steroid biosynthetic process |
GO:0008202 | TAS:Reactome | P | steroid metabolic process |
GO:0061370 | IEA:Ensembl | P | testosterone biosynthetic process |
KEGG Pathway Links
REACTOME Pathway Links
REACTOME Pathway ID | Description |
---|---|
REACT_11038 | Pregnenolone biosynthesis |
Domain Information
UniProt Annotations
Entry Information
Gene Name
steroidogenic acute regulatory protein
Protein Entry
STAR_HUMAN
UniProt ID
Species
Human
Comments
Comment Type | Description |
---|---|
Disease | Adrenal hyperplasia 1 (AH1) [MIM |
Function | Plays a key role in steroid hormone synthesis by enhancing the metabolism of cholesterol into pregnenolone. Mediates the transfer of cholesterol from the outer mitochondrial membrane to the inner mitochondrial membrane where it is cleaved to pregnenolone. |
Pathway | Steroid metabolism; cholesterol metabolism. |
Similarity | Contains 1 START domain. {ECO |
Subcellular Location | Mitochondrion. |
Subunit | May interact with TSPO. |
Tissue Specificity | Expressed in gonads, adrenal cortex and kidney. |
Web Resource | Name=Wikipedia; Note=Steroidogenic acute regulatory protein entry; URL="http://en.wikipedia.org/wiki/Steroidogenic_acute_regulatory_protein"; |
Identical and Related Proteins
Unique RefSeq proteins for LMP002045 (as displayed in Record Overview)
Protein GI | Database | Accession | Length | Protein Name |
---|---|---|---|---|
56243551 | RefSeq | NP_000340 | 285 | steroidogenic acute regulatory protein, mitochondrial |
Identical Sequences to LMP002045 proteins
Reference | Database | Accession | Length | Protein Name |
---|---|---|---|---|
GI:56243551 | DBBJ | BAG37577.1 | 285 | unnamed protein product [Homo sapiens] |
GI:56243551 | GenBank | EAW63334.1 | 285 | steroidogenic acute regulator, isoform CRA_a [Homo sapiens] |
GI:56243551 | GenBank | EAW63335.1 | 285 | steroidogenic acute regulator, isoform CRA_a [Homo sapiens] |
GI:56243551 | GenBank | ABM82732.1 | 285 | steroidogenic acute regulator [synthetic construct] |
GI:56243551 | GenBank | AHD69666.1 | 285 | Sequence 680 from patent US 8586006 |
GI:56243551 | GenBank | AIC49798.1 | 285 | STAR, partial [synthetic construct] |
Related Sequences to LMP002045 proteins
Reference | Database | Accession | Length | Protein Name |
---|---|---|---|---|
GI:56243551 | GenBank | AAC50234.1 | 285 | steroidogenic acute regulatory protein [Homo sapiens] |
GI:56243551 | GenBank | AAP36410.1 | 286 | Homo sapiens steroidogenic acute regulatory protein, partial [synthetic construct] |
GI:56243551 | GenBank | AAX37038.1 | 286 | steroidogenic acute regulator, partial [synthetic construct] |
GI:56243551 | GenBank | AAX43505.1 | 286 | steroidogenic acute regulator, partial [synthetic construct] |
GI:56243551 | GenBank | AAX43506.1 | 286 | steroidogenic acute regulator, partial [synthetic construct] |
GI:56243551 | RefSeq | XP_001170375.1 | 285 | PREDICTED: steroidogenic acute regulatory protein, mitochondrial isoform X2 [Pan troglodytes] |