英文名称Rabbit Anti-StAR antibody
中文名称类固醇激素合成急性调节蛋白抗体
别 名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. 、
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human,Mouse,Rat (predicted: Cow,Dog,Horse)
产品应用WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=3μg /Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量32kDa
细胞定位细胞浆 线粒体
性 状Liquid
浓 度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human StAR/StARD1: 101-200/285
亚 型IgG
纯化方法affinity purified by Protein A
缓 冲 液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMedPubMed
产品介绍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.
SWISS:
P49675
Gene ID:
6770
