Anti-ECHS1 antibody produced in rabbit
SIGMA/HPA021995 - Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution, Ab1
Synonym: Anti-Enoyl-CoA hydratase 1; Anti-Enoyl-CoA hydratase, mitochondrial; Anti-SCEH; Anti-Short chain enoyl-CoA hydratase
Product Type: Chemical
Enhanced Validation-By Independent Antibodies Immunohistochemical staining of human gastrointestinal, heart muscle, kidney and liver using Anti-ECHS1 antibody HPA021995 (A) shows similar protein distribution across tissues to independent antibody HPA022476 (B).
Enhanced Validation-By Independent Antibodies Western blot analysis using Anti-ECHS1 antibody HPA021995 (A) shows similar pattern to independent antibody HPA022476 (B).
Immunohistochemistry Immunohistochemical staining of human liver shows strong granular cytoplasmic positivity in hepatocytes.
Immunohistochemistry Immunohistochemical staining of human heart muscle shows strong granular cytoplasmic positivity in cardiomyocytes.
Immunohistochemistry Immunohistochemical staining of human kidney shows strong granular cytoplasmic positivity in cells in tubules.
Immunohistochemistry Immunohistochemical staining of human duodenum shows strong granular cytoplasmic positivity in glandular cells.
Immunofluorescence Immunofluorescent staining of human cell line A-431 shows localization to mitochondria.
Western Blotting Lane 1: NIH-3T3 cell lysate (Mouse embryonic fibroblast cells) Lane 2: NBT-II cell lysate (Rat Wistar bladder tumour cells)
| antibody form | affinity isolated antibody |
| antibody product type | primary antibodies |
| biological source | rabbit |
| clone | polyclonal |
| conjugate | unconjugated |
| enhanced validation | independent Learn more about Antibody Enhanced Validation  |
| form | buffered aqueous glycerol solution |
| immunogen sequence | ANFEYIIAEKRGKNNTVGLIQLNRP |
| product line | Prestige Antibodies® Powered by Atlas Antibodies |
| Quality Level | 100 |
| shipped in | wet ice |
| species reactivity | rat, mouse, human |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | immunoblotting: 0.04-0.4 μg/mL |
| immunofluorescence: 0.25-2 μg/mL | |
| immunohistochemistry: 1:500-1:1000 | |
| UniProt accession no. | P30084  |
| Application: | All Prestige Antibodies Powered by Atlas Antibodies are developed and validated by the Human Protein Atlas (HPA) project and as a result, are supported by the most extensive characterization in the industry. The Human Protein Atlas project can be subdivided into three efforts: Human Tissue Atlas, Cancer Atlas, and Human Cell Atlas. The antibodies that have been generated in support of the Tissue and Cancer Atlas projects have been tested by immunohistochemistry against hundreds of normal and disease tissues and through the recent efforts of the Human Cell Atlas project, many have been characterized by immunofluorescence to map the human proteome not only at the tissue level but now at the subcellular level. These images and the collection of this vast data set can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. We also provide Prestige Antibodies® protocols and other useful information. |
| Biochem/physiol Actions: | ECHS1 (Enoyl CoA hydratase, short chain, 1, mitochondrial) is involved 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. |
| 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. |
| Features and Benefits: | Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section. Every Prestige Antibody is tested in the following ways: • IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues. • Protein array of 364 human recombinant protein fragments. |
| 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, fibroblasts and muscles. |
| Immunogen: | Enoyl-CoA hydratase, mitochondrial Precursor recombinant protein epitope signature tag (PrEST) |
| Legal Information: | Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany |
| Other Notes: | Corresponding Antigen APREST75487 |
| Physical form: | Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 1 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |