ELK Biotechnology NPC1 rabbit pAb

제품명

NPC1 rabbit pAb

제품 설명

NPC1 단백질에 대한 토끼 폴리클로날 항체입니다. 이 항체는 인간 NPC1 단백질의 1073–1123 아미노산 서열에서 유래한 합성 펩타이드를 면역원으로 사용하여 제작되었습니다. 주로 Western blot(WB) 분석에 적합하며, 인간과 마우스 시료에서 반응성을 보입니다.

제품 정보

Background

This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome—the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolyzed and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by overaccumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments. [provided by RefSeq, Aug 2009]