Human ECHS1 Matched Antibody Pair set for ELISA useCapture Antibody: Rabbit purified polyclonal anti-ECHS1Detection Antibody: Mouse monoclonal anti-ECHS1, IgG1 Kappa
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Antigen Detail | |
|---|---|
| Target | ECHS1 |
| Description | 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. ECHS1 suppresses renal cell carcinoma development through inhibiting mTOR signaling activation. In clear cell renal cell carcinoma (ccRCC) ECHS1 downregulation induced fatty acid (FA) and branched-chain amino acid (BCAA) accumulation, which inhibited AMPK-promoted expression of GATA3, a transcriptional activator of ECHS1. |
| Species Reactivity | Human |
Capture Antibody | |
|---|---|
| Capture Clonality | Polyclonal |
| Host Species | Rabbit |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4, containing 0.1% Proclin 300 |
| Application | ELISA |
Detection Antibody | |
|---|---|
| Det-Clonality | Monoclonal |
| Host Species | Mouse |
| Conjugation | Unconjugated |
| Clone | RD1065 |
| Isotype | IgG1, kappa |
| Purification | Affinity chromatography |
| Buffer | PBS, pH 7.4 |
General Product Property | |
|---|---|
| Product Overview | Human ECHS1 Matched Antibody Pair set for ELISA use Capture Antibody: Rabbit purified polyclonal anti-ECHS1 Detection Antibody: Mouse monoclonal anti-ECHS1, IgG1 Kappa |
| Specificity | Validated for detecting natural and recombinant human ECHS1 |
| Research Area | Subcellular Markers |
| Application | ELISA |
| Application Notes | Optimal dilutions should be determined experimentally by the user. |
| Form | Lyophilized |
| Storage | Avoid repeated freeze/thaw cycles. Store at 2-8°C for short term. Aliquot and store at -20°C for long term storage. |
| Size | 1 Set |
| Sample Type | Serum; Supernatant; Urine; Cell lysate; Plasma; Tissue Homogenates |
| Usage Statement | Available for Research Use Only. Not for use in therapeutic or diagnostic applications. |
Target Overview | |
|---|---|
| Gene Symbol | ECHS1 |
| Full Name | Enoyl-CoA Hydratase, Short Chain 1 |
| Background | 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. |
| Alternative Names | Enoyl Coenzyme A Hydratase; Short Chain; 1; Mitochondrial |
| Gene ID | 1892 |
| Uniprot ID | P30084 |
| GeneCards | ECHS1 |
| HGNC | 3151 |
| Ensembl | ENSG00000127884 |
| OMIM | 602292 |
| Involvement in Disease | Diseases associated with ECHS1 include Mitochondrial Short-Chain Enoyl-Coa Hydratase 1 Deficiency and Leigh Syndrome With Leukodystrophy. |
| Function | Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate. |
| Related Pathways | Its related pathways are beta-Alanine metabolism (KEGG) and Lysine degradation. |
For Research Use Only.
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