产品详情
简单介绍：
Anti-ECHS1 Antibody
详情介绍：
Overview
| Name: | Anti-ECHS1 Antibody See all ECHS1 primary antibodies
|
| Description: | Rabbit polyclonal antibody to ECHS1 |
| Specificity: | The antibody detects endogenous levels of total ECHS1 protein. |
| Applications: | WB, IHC |
| Reactivity: | Human |
| Immunogen: | Full length fusion protein |
| Host: | Rabbit |
| Clonality: | Polyclonal |
| Conjugate: | Unconjugated |
| Purification: | Antigen affinity purification. |
| Concentration: | 1.6mg / ml |
| Formulation: | Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol. |
| Storage: | Store at -20?C |
Target
| 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. |
| Tissue Specificity: | Liver, fibroblast, muscle. Barely detectable in spleen and kidney. |
| Involvement in Disease: | Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency: A severe, autosomal recessive inborn error affecting valine metabolism. Disease features include brain lesions in the basal ganglia, neurodegeneration, delayed psychomotor development, hypotonia, spasticity, and increased lactic acid in serum and cerebral serum fluid. |
| Sequence Similarities: | Belongs to the enoyl-CoA hydratase/isomerase family. |
| Cellular Location: | Mitochondrion matrix. |
| Database Links: |
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| Synonyms: | |
| Information: | Target information shown above is from the UniProt Consortium. |
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