• 靶点：
• STAR

• 简介：
• >>Ovarian steroidogenesis;>>Aldosterone synthesis and secretion;>>Cortisol synthesis and secretion;>>Cushing syndrome;>>Cholesterol metabolism

• 基因名称：
• STAR STARD1

• 蛋白名称：
• Steroidogenic acute regulatory protein, mitochondrial (StAR) (START domain-containing protein 1) (StARD1)

• Human Gene Id：
• 6770

• Human Swiss Prot No：
• P49675

• Mouse Swiss Prot No：
• P51557

• Rat Swiss Prot No：
• P97826

• 免疫原：
• Synthesized peptide derived from part region of human protein

• 特异性：
• STAR Polyclonal Antibody detects endogenous levels of protein.

• 组成：
• Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

• 来源：
• Polyclonal, Rabbit,IgG

• 稀释：
• WB 1:500-2000 ELISA 1:5000-20000

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

• 储存：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• 实测条带：
• 31kD

• 背景：
• 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],

• 功能：
• disease:Defects in STAR are a cause of congenital lipoid adrenal hyperplasia (CLAH) [MIM:201710]; also called lipoid CAH. CLAH is the most severe form of adrenal hyperplasia. This autosomal recessive and potentially lethal condition includes the 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. CLAH is a rare disease, except in Japan and Korea where it accounts for a significant percentage of cases of congenital adrenal hyperplasia.,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

• 细胞定位：
• Mitochondrion .

• 组织表达：
• Expressed in gonads, adrenal cortex and kidney.

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