The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
% SDS-PAGE. ab115707 was purified using conventional chromatography techniques.
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Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
pH: 8.00 Constituents: 0.32% Tris HCl, 20% Glycerol, 0.03% DTT
Luteinizing hormone induced protein
Mitochondrial steroid acute regulatory protein
StAR related lipid transfer (START) domain containing 1
START domain containing 1
START domain containing protein 1
START domain-containing protein 1
Steroid acute regulatory protein
Steroidogenic acute regulator
Steroidogenic acute regulatory protein
Steroidogenic acute regulatory protein mitochondrial
機能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.
組織特異性Expressed in gonads, adrenal cortex and kidney.
パスウェイSteroid metabolism; cholesterol metabolism.
関連疾患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 renin 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.