Anti-ECHAS1 Antibody Picoband® 31.0550 Vervoeging

Enoyl Coenzyme A hydratase, short chain, 1, mitochondrial, also known as ECHS1, is a human gene. 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.

Vascular endothelial growth factor B; VEGF-B; VEGF-related factor; VRF; VEGFB; VRF

ECHS1

1892

P30084

Human, Mouse, Rat

No cross-reactivity with other proteins.

E.coli-derived human ECHS1 recombinant protein (Position: R12-Q290) .

Expressed in all tissues except liver. Highest levels found in heart, skeletal muscle and pancreas.

Flow Cytometry

Angiogenesis, Cancer, Cardiovascular, Growth Factors, Growth Factors/Hormones, Invasion/Microenvironment, Neurogenesis, Neurology Process, Neuroscience, Signal Transduction

Immunogen affinity purified.

Liquid

Growth factor for endothelial cells. VEGF-B167 binds heparin and neuropilin-1 whereas the binding to neuropilin-1 of VEGF-B186 is regulated by proteolysis.

1. Fitzsimons, P. E., Alston, C. L., Bonnen, P. E., Hughes, J., Crushell, E., Geraghty, M. T., Tetreault, M., O'Reilly, P., Twomey, E., Sheikh, Y., Walsh, R., Waterham, H. R., Ferdinandusse, S., Wanders, R. J. A., Taylor, R. W., Pitt, J. J., Mayne, P. D. Clinical, biochemical, and genetic features of four patients with short-chain enoyl-CoA hydratase (ECHS1) deficiency. Am. J. Med. Genet. 176A: 1115-1127, 2018. 2. Haack, T. B., Jackson, C. B., Murayama, K., Kremer, L. S., Schaller, A., Kotzaeridou, U., de Vries, M. C., Schottmann, G., Santra, S., Buchner, B., Wieland, T., Graf, E., and 28 others. Deficiency of ECHS1 causes mitochondrial encephalopathy with cardiac involvement. Ann. Clin. Transl. Neurol. 2: 492-509, 2015. 3. Janssen, U., Davis, E. M., Le Beau, M. M., Stoffel, W. Human mitochondrial enoyl-CoA hydratase gene (ECHS1) : structural organization and assignment to chromosome 10q26.2-q26.3. Genomics 40: 470-475, 1997.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

6

Vascular endothelial growth factor B

Secreted. Secreted but remains associated to cells or to the extracellular matrix unless released by heparin.

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.