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:0043025 IEA:Ensembl C neuronal cell body
GO:0043005 IEA:Ensembl C neuron projection
GO:0015485 IEA:Ensembl F cholesterol binding
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:0006700 TAS:Reactome P C21-steroid hormone biosynthetic process
GO:0071312 IEA:Ensembl P cellular response to alkaloid
GO:0071236 IEA:Ensembl P cellular response to antibiotic
GO:0071276 IEA:Ensembl P cellular response to cadmium ion
GO:0071320 IEA:Ensembl P cellular response to cAMP
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

KEGG Pathway ID Description
hsa04913 Ovarian steroidogenesis
ko04913 Ovarian steroidogenesis

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_11038 Pregnenolone biosynthesis

Domain Information

InterPro Annotations

Accession Description
IPR023393 START-like domain
IPR002913 START_lipid-bd_dom
IPR000799 Steroidogenic acute regulatory protein-like

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]