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Anti-ECHS1 Antibody

品牌：Antibodies

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规格：100µl

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Anti-ECHS1 Antibody

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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:	Entrez Gene: 1892?Human Omim: 602292?Human SwissProt: P30084?Human Unigene: 76394?Human
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.