ECHS1

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.
Full Name
ECHS1
Research Area
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.
Biological Process
Branched-chain amino acid catabolic process Source: Reactome
Fatty acid beta-oxidation Source: UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (ECHS1D):
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.
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Anti-ECHS1 antibodies

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Mouse Anti-ECHS1 Recombinant Antibody (3C6) (CBMAB-A2430-LY)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 3C6
Application*: SE, E
Datasheet SDS

Mouse Anti-ECHS1 (AA 13-290) Recombinant Antibody (CBFYE-0408) (CBMAB-E0721-FY)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgM, κ
Specificity: Human
Clone: CBFYE-0408
Application*: E, IP
Datasheet SDS

Mouse Anti-ECHS1 Recombinant Antibody (CBFYE-0407) (CBMAB-E0719-FY)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYE-0407
Application*: E, WB, IH, IP IF
Datasheet SDS

Mouse Anti-ECHS1 Recombinant Antibody (CF140) (V2LY-0425-LY1339)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CF140
Application*: WB, P, IP, IF
Datasheet SDS

Mouse Anti-ECHS1 Recombinant Antibody (3C6) (V2LY-0425-LY1338)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 3C6
Application*: E
Datasheet SDS

Mouse Anti-ECHS1 Recombinant Antibody (CBFYE-0408) (V2LY-0425-LY1337)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBFYE-0408
Application*: E
Datasheet SDS

Mouse Anti-ECHS1 Recombinant Antibody (CBFYE-0407) (V2LY-0425-LY1336)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYE-0407
Application*: E, WB, IH, IP IF
Datasheet SDS

ECHS1 Matched Antibody Pair (361) (APMAB-361LY)

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Target: ECHS1
Specificity: Human
Datasheet SDS

Mouse Anti-ECHS1 Recombinant Antibody (CF140) (CBMAB-FT334LY)

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Target: ECHS1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CF140
Application*: ELISA, WB, IHC, IP, IF
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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