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FLASH GENE
Symbol STAR contributors: mct - updated : 08-06-2016
HGNC name steroidogenic acute regulatory protein
HGNC id 11359
Corresponding disease
CLAH congenital lipoid adrenal hyperplasia
Location 8p11.23      Physical location : 38.000.217 - 38.008.600
Synonym name
  • start domain containing 1
  • steroidogenic acute regulator
  • StAR-related lipid transfer (START) domain containing 1
  • mitochondrial steroid acute regulatory protein
  • Synonym symbol(s) STARD1
    DNA
    TYPE functioning gene
    STRUCTURE 8.38 kb     7 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence Promoter
    text structure
  • NR5A1 binding sites upstream of the STAR gene functioning as a strong transcriptional activator
  • MAPPING cloned Y linked N status confirmed
    Physical map
    LOC392212 8 similar to ribosomal protein L23 MRPS7P1 8p11.22 mitochondrial ribosomal protein S7 pseudogene 1 FKSG2 8p11.2 apoptosis inhibitor LOC392213 8 similar to SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily e, member 1; BAF57 LOC389647 8 LOC389647 LOC389648 8 LOC389648 FLJ14299 8p11.23 hypothetical protein FLJ14299 C8orf2 8p11.2 chromosome 8 open reading frame 2 PROSC 8p11.2 proline synthetase co-transcribed homolog (bacterial) GPR124 8p11.23 G protein-coupled receptor 124 BRF2 8q11.23 BRF2, subunit of RNA polymerase III transcription initiation factor, BRF1-like RCP 8p11.22 Rab coupling protein MGC33309 8p11.23 hypothetical protein MGC33309 ASH2L 8p11.2 ash2 (absent, small, or homeotic)-like (Drosophila) STAR 8p11.2 steroidogenic acute regulatory protein LSM1 8p11.2 LSM1 homolog, U6 small nuclear RNA associated (S. cerevisiae) BAG4 8p11.23 BCL2-associated athanogene 4 KIAA0725 8p11.22 BCL2-associated athanogene 4 HTPAP 8p11.23 HTPAP protein WHSC1L1 8p12-p11.2 Wolf-Hirschhorn syndrome candidate 1-like 1 FLJ25409 8p11.22 hypothetical protein FLJ25409 FGFR1 8p12 fibroblast growth factor receptor 1 (fms-related tyrosine kinase 2, Pfeiffer syndrome) LOC389649 8 hypothetical gene supported by AK125570 LOC286140 8p11.22 similar to ring finger protein 5 TACC1 8p11 transforming, acidic coiled-coil containing protein 1 PLEKHA2 8p11.21 pleckstrin homology domain containing, family A (phosphoinositide binding specific) member 2 FLJ90724 8p11.22 hypothetical protein FLJ90724 BLP1 8p11.22 BBP-like protein 1 ADAM9 8p12 a disintegrin and metalloproteinase domain 9 (meltrin gamma) ADAM32 8p11.22 a disintegrin and metalloprotease domain 32 ADAM5 8p11.22 a disintegrin and metalloproteinase domain 5 ADAM3A 8p21-p12 a disintegrin and metalloproteinase domain 3a (cyritestin 1) ADAM18 8p11.21 a disintegrin and metalloproteinase domain 18 ADAM2 8p11.2 a disintegrin and metalloproteinase domain 2 (fertilin beta) INDO 8p11.2 indoleamine-pyrrole 2,3 dioxygenase
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    7 - 2695 32.1 285 - 1995 7548191
    EXPRESSION
    Type restricted
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Endocrineadrenal glandcortex   
    Nervousbrain   highly
    Reproductivefemale systemplacenta   
     female systemovarycorpus luteum  
     male systemtestis   
    Urinarykidney    
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Epithelialsecretoryglandularendocrine 
    cell lineage
    cell lines oligodendrogliomas and malignant glial tumor cell lines
    fluid/secretion
    at STAGE
    physiological period pregnancy
    Text placenta
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • one start domain
  • HOMOLOGY
    Homologene
    FAMILY
    CATEGORY transport carrier
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,interspace
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,cytosolic
    text stabilized at the outer mitochondrial membrane by cholesterol to initiate its massive import into mitochondria
    basic FUNCTION
  • increasing the flux of cholesterol into mitochondria and enhanced resorption of cholesterol from sterol-rich donor membranes
  • also involved in the activity of the mitochondrial cholesterol metabolizing enzymes
  • mediating rapid increase in pregnenolone synthesis by tropic hormones
  • mitochondrial matrix protein, acts on the outer mitochondrial membrane (OMM) to facilitate the movement of cholesterol from the outer to inner mitochondrial membrane via an unknown mechanism
  • acting on the outside of mitochondria to stimulate the steroidogenesis regulated by CEBPB and repressed by DAX1
  • plays an important role in the maintenance of intracellular lipid homeostasis
  • plays an important role in macrophage and foam cell apoptotic processing, which may provide a potential method for preventing atherosclerosis
  • mediates the transport of cholesterol from the outer to the inner mitochondrial membrane, the process of which is the rate-limiting step for steroidogenesis
  • STAR and STARD6 bind cholesterol, but no cholesterol binding could be detected for STARD5
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    steroidogenesis, regulated and rate limiting step
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • stimulating cholesterol 27 hydroxylase activity
  • STAR xpression level increased by STS
  • cholesterol, 25-hydroxycholesterol, phosphatidylcholine, phosphatidylethanolamine and ceramides are ligands for STAR/STARD3/STARD5, STARD5, PCTP/STARD10, STARD10 and COL4A3BP, respectively
  • differential cholesterol binding of the two most closely related START domain proteins STAR and STARD3
  • interacting with UBE2I and FOXL2 (UBE2I-mediated sumoylation at lysine 25 of FOXL2 is required for transcriptional repression of the STAR gene and may be responsible for controlling the development of ovarian follicles)
  • both PDE8A and PDE8B play essential roles to maintain low cAMP levels, thereby suppressing resting steroidogenesis by keeping cholesterol-ester hydrolase (CEH)/hormone-sensitive lipase (HSL) inactive and STAR protein expression low
  • AKAP1 interacts with STAR mRNA in a dibutyryl-cAMP-stimulated human steroidogenic adrenocortical carcinoma cell line
  • to prevent mitochondrial impairment, STAR proteolysis is executed by at least 2 mitochondrial proteases, ie, the matrix LONP1 and the inner membrane complexes of the metalloproteases AFG3L2 and SPG7/paraplegin
  • GPLD1 mediates STAR protein and aldosterone synthase (CYP11B2) expression via increasing the phosphorylation (activation) of their regulatory transcription factors
  • cell & other
    REGULATION
    repressed by sterol depletion (methyl beta-cyclodextrin), repressing STAR expression
    Other regulated by its rate of mitochondrial import (inhibition of the activity in mitochondrial importation)
    ASSOCIATED DISORDERS
    corresponding disease(s) CLAH
    related resource MITOP database
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in malignant glial tumors in growth
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS