L2HGDH

L2HGDH is a FAD-dependent enzyme that oxidizes L-2-hydroxyglutarate to alpha-ketoglutarate in a variety of mammalian tissues. Mutations in this gene lead to L-2-hydroxyglutaric aciduria, a rare autosomal recessive neurometabolic disorder resulting in moderate to severe cognitive disability.

Full Name

L2HGDH

Biological Process

2-oxoglutarate metabolic processTAS:Reactome
Small molecule metabolic processManual Assertion Based On ExperimentIDA:HGNC-UCL

Cellular Location

Mitochondrion

Involvement in disease

L-2-hydroxyglutaric aciduria (L2HGA):
A rare autosomal recessive disorder clinically characterized by mild psychomotor delay in the first years of life, followed by progressive cerebellar ataxia, dysarthria and moderate to severe mental retardation. Diagnosis is based on the presence of an excess of L-2-hydroxyglutaric acid in urine, blood and cerebrospinal fluid.

Anti-L2HGDH antibodies

Fig.3 Signaling pathways in cancers. (Creative Biolabs Authorized)

Fig.4 Protocols troubleshootings & guides. (Creative Biolabs Authorized)

Mouse Anti-L2HGDH Recombinant Antibody (CBYJL-1033) (CBMAB-L0445-YJ)

Datasheet

SDS

Target: L2HGDH

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human, Pig

Clone: CBYJL-1033

Application*: E, IH, WB

Mouse Anti-L2HGDH Recombinant Antibody (A852) (CBMAB-AP11298LY)

Datasheet

SDS

Target: L2HGDH

Host: Mouse

Antibody Isotype: IgG2a

Specificity: Human

Clone: A852

Application*: ELISA, IHC, WB

More Infomation

For Research Use Only. Not For Clinical Use.

(P): Predicted

* Abbreviations

IFImmunofluorescence

IHImmunohistochemistry

IPImmunoprecipitation

WBWestern Blot

EELISA

MMicroarray

CIChromatin Immunoprecipitation

FFlow Cytometry

FNFunction Assay

IDImmunodiffusion

RRadioimmunoassay

TCTissue Culture

GSGel Supershift

NNeutralization

BBlocking

AActivation

IInhibition

DDepletion

ESELISpot

DBDot Blot

MCMass Cytometry/CyTOF

CTCytotoxicity

SStimulation

AGAgonist

APApoptosis

IMImmunomicroscopy

BABioassay

CSCostimulation

EMElectron Microscopy

IEImmunoelectrophoresis

PAPeptide Array

ICImmunocytochemistry

PEPeptide ELISA

MDMeDIP

SHIn situ hybridization

IAEnzyme Immunoassay

SEsandwich ELISA

PLProximity Ligation Assay

ECELISA(Cap)

EDELISA(Det)

BIBioimaging

IOImmunoassay

LFLateral Flow Immunoassay

LALuminex Assay

CImmunohistochemistry-Frozen Sections

PImmunohistologyp-Paraffin Sections

ISIntracellular Staining for Flow Cytometry

MSElectrophoretic Mobility Shift Assay

RIRNA Binding Protein Immunoprecipitation (RIP)