Anti-ECHS1 Antibody Picoband®, APC Conjugate

• 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.

• Description:

  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.

• 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, -
  

• Subcellular Location:

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