Anti-ECHS1 antibody produced in mouse

NA.41

The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature. (provided by RefSeq)

ECHS1 (NP_004083.2, 1 a.a. ~ 290 a.a) full-length human protein.

Sequence
MAALRVLLSCARGPLRPPVRCPAWRPFASGANFEYIIAEKRGKNNTVGLIQLNRPKALNALCDGLIDELNQALKIFEEDPAVGAIVLTGGDKAFAAGADIKEMQNLSFQDCYSSKFLKHWDHLTQVKKPVIAAVNGYAFGGGCELAMMCDIIYAGEKAQFAQPEILIGTIPGAGGTQRLTRAVGKSLAMEMVLTGDRISAQDAKQAGLVSKICPVETLVEEAIQCAEKIASNSKIVVAMAKESVNAAFEMTLTEGSKLEKKLFYSTFATDDRKEGMTAFVEKRKANFKDQ

Anti-ECHS1 antibody produced in mouse is suitable for indirect immunofluorescence and western blot applications.

ECHS1 (Enoyl CoA hydratase, short chain, 1, mitochondrial) performs in the mitochondrial β-oxidation of fatty acid by catalyzing the second step of the process i.e. 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, valine. Study reports that heterozygous mutation in ECHS1 causes Leigh syndrome with hypotonia, metabolic acidosis, and developmental delay symptoms.

Solution in phosphate buffered saline, pH 7.4

100

mouse

unconjugated

purified immunoglobulin

primary antibodies

polyclonal

buffered aqueous solution

antigen ~31.4 kDa

human

indirect immunofluorescence: suitable
western blot: 1 μg/mL

NM_004092.2

P30084

dry ice

−20℃

human ... ECHS1(1892)

13 - Non Combustible Solids

WGK 1