Application

Anti-ECHS1 (AB1) antibody produced in rabbit is suitable for western blotting at a concentration of 5.0µg/ml.

Biochem/physiol Actions

Enoyl CoA hydratase, short chain, 1 (ECHS1) is a mitochondrial enzyme that functions in the fatty acid β-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) to L-3-hydroxyacyl-CoAs. It also participates in different metabolic pathways involving fatty acids and amino acids. Study reports that heterozygous mutation in ECHS1 causes Leigh syndrome with hypotonia, metabolic acidosis, and developmental delay symptoms.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

General description

ECHS1 (Enoyl CoA hydratase, short chain, 1, mitochondrial) is structurally located in chromosome 10q26.2-q26.3 region consisting of eight exons, with exons I and VIII with the 5′- and 3′-untranslated regions. It has a GC-rich 5′-flanking region with multiple copies of the SP1 binding motive. Its expressions have been found in the human liver, fibroblast and muscle.

Immunogen

Synthetic peptide directed towards the middle region of human ECHS1

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Sequence

Synthetic peptide located within the following region: RAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIA