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Product Name Anti-StAR/RBITC Chinese Name 罗丹明(RBITC)标记的促黄体激素诱导蛋白抗体 Alias StARD1; Cholesterol trafficker; Luteinizing hormone induced protein; Mitochondrial steroid acute regulatory protein; StAR related lipid transfer (START) domain containing 1; StARD1; START domain containing protein 1; Steroidogenic Acute Regulatory Protein; Steroidogenic acute regulatory protein mitochondrial; STAR_HUMAN. Research Area Tumour immunology transcriptional regulatory factor Kinases and Phosphatases Mitochondrion Immunogen Species Rabbit Clonality Polyclonal React Species Human, Mouse, Rat, (predicted: Dog, Cow, Horse, ) Applications IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 32kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human StAR/StARD1 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
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].
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.
Subunit:
May interact with TSPO.
Subcellular Location:
Mitochondrion.
DISEASE:
Defects in STAR are the cause of adrenal hyperplasia type 1 (AH1) [MIM:201710]. The most severe form of adrenal hyperplasia. It is a condition characterized by onset of profound adrenocortical insufficiency shortly after birth, hyperpigmentation reflecting increased production of pro-opiomelanocortin, elevated plasma rennin activity as a consequence of reduced aldosterone synthesis, and male pseudohermaphroditism resulting from deficient fetal testicular testosterone synthesis. Affected individuals are phenotypic females irrespective of gonadal sex, and frequently die in infancy if mineralocorticoid and glucocorticoid replacement are not instituted.
Similarity:
Contains 1 START domain.
Database links:Entrez Gene: 6770 Human
Entrez Gene: 20845 Mouse
Omim: 600617 Human
SwissProt: P49675 Human
SwissProt: P51557 Mouse
Unigene: 521535 Human
Unigene: 293314 Mouse
Unigene: 11399 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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