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Anti-ECHS1 Antibody

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产品名称: Anti-ECHS1 Antibody
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简单介绍

Anti-ECHS1 Antibody


Anti-ECHS1 Antibody  的详细介绍
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
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:
  • Entrez Gene: 1892?Human
  • Omim: 602292?Human
  • SwissProt: P30084?Human
  • Unigene: 76394?Human
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    Synonyms:
  • C80529 Antibody
  • EC 4.2.1.17 Antibody
  • ECHS 1 Antibody
  • ECHS1 Antibody
  • Enoyl CoA hydratase 1 Antibody
  • Enoyl CoA hydratase mitochondrial Antibody
  • Enoyl CoA hydratase short chain 1 mitochondrial Antibody
  • Enoyl Coenzyme A hydratase short chain 1 Antibody
  • Enoyl Coenzyme A hydratase short chain 1 mitochondrial Antibody
  • Mitochondrial short chain enoyl coenzyme A hydratase 1 Antibody
  • OTTHUMP00000020811 Antibody
  • SCEH Antibody
  • Short chain enoyl CoA hydratase Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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