ELK Biotechnology ECHA rabbit pAb

제품명

ECHA rabbit pAb

제품 개요

ECHA rabbit pAb는 미토콘드리아 트라이펑셔널 단백질 알파 서브유닛(ECHA)을 인식하는 폴리클로날 항체입니다. 이 단백질은 긴 사슬 지방산의 미토콘드리아 β-산화의 마지막 세 단계를 촉매하는 효소 복합체의 일부로, 에너지 대사 및 관련 질환 연구에 활용됩니다.

제품 정보

Background

This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. (RefSeq, Jul 2008)