Anti-ECHS1 Antibody Picoband®, Dylight594 Conjugate

Boster Bio Anti-ECHS1 Antibody Picoband® catalog # A04508-1. Tested in ELISA, Flow Cytometry, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Product Specifications

Background

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.

Synonyms

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

Gene Name

vascular endothelial growth factor B

Gene ID

1892

UniProt

P30084

Host

Rabbit

Reactivity

Human,Mouse,Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

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

Clonality

Polyclonal

Tissue Specificity

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

Applications

WB,IHC,ICC,IF,Flow Cytometry,ELISA

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Function

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.

Components

Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

References & Citations

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.

Storage Conditions

At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Observed Molecular Weight

29 kDa

Fragment

Rabbit IgG

Applications Notes

Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human, Rat Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human Immunofluorescence, 5 μg/ml, Human Flow Cytometry (Fixed), 1-3 μg/1x10^6 cells, Human ELISA, 0.1-0.5 μg/ml, -