HMGCL

The protein encoded by this gene belongs to the HMG-CoA lyase family. It is a mitochondrial enzyme that catalyzes the final step of leucine degradation and plays a key role in ketone body formation. Mutations in this gene are associated with HMG-CoA lyase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2009]
Full Name
3-Hydroxymethyl-3-Methylglutaryl-CoA Lyase
Function
Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism.
Biological Process
Ketone body biosynthetic process Source: UniProtKB
Leucine catabolic process Source: GO_Central
Lipid metabolic process Source: BHF-UCL
Mitochondrion organization Source: Ensembl
Cellular Location
Mitochondrion matrix; Peroxisome. Unprocessed form is peroxisomal.
Involvement in disease
3-hydroxy-3-methylglutaryl-CoA lyase deficiency (HMGCLD):
An autosomal recessive disease affecting ketogenesis and L-leucine catabolism. The disease usually appears in the first year of life after a fasting period and its clinical acute symptoms include vomiting, seizures, metabolic acidosis, hypoketotic hypoglycemia and lethargy. These symptoms sometimes progress to coma, with fatal outcome in some cases.
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Anti-HMGCL antibodies

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Mouse Anti-HMGCL Recombinant Antibody (4F4-D1) (CBMAB-A3974-LY)

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Target: HMGCL
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 4F4-D1
Application*: WB, E
Datasheet SDS

Mouse Anti-HMGCL Recombinant Antibody (CBFYH-1545) (CBMAB-H2520-FY)

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Target: HMGCL
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYH-1545
Application*: E, IF, IP
Datasheet SDS

Mouse Anti-HMGCL Recombinant Antibody (9F5AB9) (CBMAB-H2517-FY)

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Target: HMGCL
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 9F5AB9
Application*: IP, IF, E
Datasheet SDS

Rabbit Anti-HMGCL Recombinant Antibody (
22H11) (V2LY-0725-LY990)

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Target: HMGCL
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 22H11
Application*: E, WB, IH, F
Datasheet SDS

HMGCL Matched Antibody Pair (560) (APMAB-560LY)

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Target: HMGCL
Specificity: Human
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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