Catalog No.
YHK44501
Expression system
E. coli
Species
Homo sapiens (Human)
Protein length
Asp50-Leu463
Predicted molecular weight
47.81 kDa
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>90% as determined by SDS-PAGE.
Accession
Q9H9P8
Applications
ELISA, Immunogen, SDS-PAGE, WB, Bioactivity testing in progress
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution. A copy of datasheet will be provided with the products, please refer to it for details.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
C14orf160, L2HGDH, Duranin, L-2-hydroxyglutarate dehydrogenase, mitochondrial
Transsulfuration pathway activation attenuates oxidative stress and ferroptosis in sickle primary erythroblasts and transgenic mice., PMID:39762627
Non-Susceptibility Gene Variants in Head and Neck Paragangliomas., PMID:39684472
Combined Multi-Omics Analysis Reveals the Potential Role of ACADS in Yak Intramuscular Fat Deposition., PMID:39201818
Renal L-2-hydroxyglutarate dehydrogenase activity promotes hypoxia tolerance and mitochondrial metabolism in Drosophila melanogaster., PMID:39182840
l-2-Hydroxyglutarate contributes to tumor radioresistance through regulating the hypoxia-inducible factor-1α signaling pathway., PMID:39012048
Focal dystonia in an adult with L-2- hydroxyglutaric aciduria., PMID:38955445
L-2-hydroxyglutaric aciduria: a report of clinical, radiological, and genetic characteristics of two siblings from Egypt., PMID:38795053
Late-onset cerebellar ataxia and a new frameshift L2HGDH mutation in a Chinese adult with L-2-hydroxyglutaric aciduria: a case report., PMID:38703293
L-2 hydroxyglutaric aciduria: report of a Mexican-Mayan patient with the mutation c.569C>T and response to vitamin supplements and levocarnitine., PMID:38464914
Structure and biochemical characterization of l-2-hydroxyglutarate dehydrogenase and its role in the pathogenesis of l-2-hydroxyglutaric aciduria., PMID:37995940
High genetic heterogeneity of leukodystrophies in Iranian children: the first report of Iranian Leukodystrophy Registry., PMID:37597066
Loss of function variants in L2HGDH gene causing L-2-hydroxyglutaric aciduria., PMID:37378753
Glutaric aciduria and L-2-hydroxyglutaric aciduria: Clinical and molecular findings of 35 patients from Turkey., PMID:37275239
Nrf2 sensitizes ferroptosis through l-2-hydroxyglutarate-mediated chromatin modifications in sickle cell disease., PMID:37267508
Mitochondria and cytochrome components released into the plasma of severe COVID-19 and ICU acute respiratory distress syndrome patients., PMID:37031181
L-2hydroxyglutaric acid rewires amino acid metabolism in colorectal cancer via the mTOR-ATF4 axis., PMID:36879117
A novel homozygous missense mutation in L-2-HGA gene: A case report., PMID:36610237
Renal oncometabolite L-2-hydroxyglutarate imposes a block in kidney tubulogenesis: Evidence for an epigenetic basis for the L-2HG-induced impairment of differentiation., PMID:36133305
In Silico Analysis of the L-2-Hydroxyglutarate Dehydrogenase Gene Mutations and Their Biological Impact on Disease Etiology., PMID:35456504
Signaling metabolite L-2-hydroxyglutarate activates the transcription factor HIF-1α in lipopolysaccharide-activated macrophages., PMID:34929172
A novel protein truncating mutation in L2HGDH causes L-2-hydroxyglutaric aciduria in a consanguineous Pakistani family., PMID:34719772
An IDH-independent mechanism of DNA hypermethylation upon VHL inactivation in cancer., PMID:34494499
Attention deficit hyperactivity disorder: a rare clinical presentation of L-2-hydroxyglutaric aciduria., PMID:34330727
L2HGDH Missense Variant in a Cat with L-2-Hydroxyglutaric Aciduria., PMID:34062805
Oncometabolite L-2-hydroxyglurate directly induces vasculogenic mimicry through PHLDB2 in renal cell carcinoma., PMID:33320958
HIF1α is not a target of 14q deletion in clear cell renal cancer., PMID:33077781
Teleological role of L-2-hydroxyglutarate dehydrogenase in the kidney., PMID:32928875
[A pedigree of L-2-hydroxyglutaric aciduria including 3 patients caused by homozygous L2HGDH variant]., PMID:32619268
MYC Regulation of D2HGDH and L2HGDH Influences the Epigenome and Epitranscriptome., PMID:32101699
LCN2-interacting proteins and their expression patterns in brain tumors., PMID:31233712
Ascorbic acid-induced TET activation mitigates adverse hydroxymethylcytosine loss in renal cell carcinoma., PMID:30702441
Two novel L2HGDH mutations identified in a rare Chinese family with L-2-hydroxyglutaric aciduria., PMID:30217188
Biochemical and Epigenetic Insights into L-2-Hydroxyglutarate, a Potential Therapeutic Target in Renal Cancer., PMID:30108105
A Drosophila model of combined D-2- and L-2-hydroxyglutaric aciduria reveals a mechanism linking mitochondrial citrate export with oncometabolite accumulation., PMID:30108060
A novel compound heterozygous mutation of the L2HGDH gene in a Chinese boy with L-2-hydroxyglutaric aciduria: case report and literature review., PMID:29980873
Muscle Weakness, Cardiomyopathy, and L-2-Hydroxyglutaric Aciduria Associated with a Novel Recessive SLC25A4 Mutation., PMID:29654543
Identification of novel L2HGDH mutation in a large consanguineous Pakistani family- a case report., PMID:29458334
[L-2-hydroxyglutaric aciduria caused by a new mutation in the L2HGDH gene]., PMID:28617386
L2hgdh Deficiency Accumulates l-2-Hydroxyglutarate with Progressive Leukoencephalopathy and Neurodegeneration., PMID:28137912
[Analysis of L2HGDH gene mutation in a patient with 2-hydroxyglutaric aciduria]., PMID:26829733
Plants Possess a Cyclic Mitochondrial Metabolic Pathway similar to the Mammalian Metabolic Repair Mechanism Involving Malate Dehydrogenase and l-2-Hydroxyglutarate Dehydrogenase., PMID:26203119
White matter abnormalities in an adult patient with l-2-hydroxyglutaric aciduria., PMID:25982940
A mouse model of L-2-hydroxyglutaric aciduria, a disorder of metabolite repair., PMID:25763823
Successive distinct high-grade gliomas in L-2-hydroxyglutaric aciduria., PMID:25338511
Clinical, neuroimaging, and genetic features of L-2-hydroxyglutaric aciduria in Arab kindreds., PMID:24894778
Founder effect confirmation of c.241A>G mutation in the L2HGDH gene and characterization of oxidative stress parameters in six Tunisian families with L-2-hydroxyglutaric aciduria., PMID:24573090
[L-2-hydroxyglutaric aciduria: report on two cases]., PMID:24321868
A L2HGDH initiator methionine codon mutation in a Yorkshire terrier with L-2-hydroxyglutaric aciduria., PMID:22834903
For research use only. Not for human or drug use.
