Cell Biology Antibodies 11
Anti-L2HGDH Antibody (CAB7996)
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- SKU:
- CAB7996
- Product Type:
- Antibody
- Applications:
- WB
- Applications:
- IHC
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Description
| Antibody Name: | L2HGDH Rabbit Polyclonal Antibody |
| Antibody SKU: | CAB7996 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-220 of human L2HGDH (NP_079160.1). |
| Application: | WB IHC |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human, Mouse |
| Positive Samples: | Mouse kidney, Mouse heart |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-220 of human L2HGDH (NP_079160.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MVPA LRYL VGAC GRAR GLFA GGSP GACG FASG RPRP LCGG SRSA STSS FDIV IVGG GIVG LASA RALI LRHP SLSI GVLE KEKD LAVH QTGH NSGV IHSG IYYK PESL KAKL CVQG AALL YEYC QQKG ISYK QCGK LIVA VEQE EIPR LQAL YEKG LQNG VPGL RLIQ QEDI KKKE PYCR GLMA IDCP HTGI VDYR QVAL SFAQ DFQE AGGS VLTN FEVK |
| Gene ID: | 79944 |
| Uniprot: | Q9H9P8 |
| Cellular Location: | Mitochondrion |
| Calculated MW: | 48kDa/50kDa |
| Observed MW: | 40kDa |
| Synonyms: | L2HGDH, C14orf160, L2HGA | ||
| Background: | This gene encodes L-2-hydroxyglutarate dehydrogenase, a FAD-dependent enzyme that oxidizes L-2-hydroxyglutarate to alpha-ketoglutarate in a variety of mammalian tissues. Mutations in this gene cause L-2-hydroxyglutaric aciduria, a rare autosomal recessive neurometabolic disorder resulting in moderate to severe mental retardation. |
| UniProt Protein Function: | L2HGDH: Defects in L2HGDH are the cause of L-2-hydroxyglutaric aciduria (L2HGA). L2HGA is 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. Belongs to the L2HGDH family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Carbohydrate Metabolism - butanoate; EC 1.1.99.2; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 14q21.3 Cellular Component: integral to membrane; mitochondrial inner membrane; mitochondrion Molecular Function:2-hydroxyglutarate dehydrogenase activity Biological Process: 2-oxoglutarate metabolic process; cellular protein metabolic process Disease: L-2-hydroxyglutaric Aciduria |
| NCBI Summary: | This gene encodes L-2-hydroxyglutarate dehydrogenase, a FAD-dependent enzyme that oxidizes L-2-hydroxyglutarate to alpha-ketoglutarate in a variety of mammalian tissues. Mutations in this gene cause L-2-hydroxyglutaric aciduria, a rare autosomal recessive neurometabolic disorder resulting in moderate to severe mental retardation. [provided by RefSeq, Jul 2008] |
| UniProt Code: | Q9H9P8 |
| NCBI GenInfo Identifier: | 13376331 |
| NCBI Gene ID: | 79944 |
| NCBI Accession: | NP_079160.1 |
| UniProt Secondary Accession: | Q9H9P8,Q9BRR1, |
| UniProt Related Accession: | Q9H9P8 |
| Molecular Weight: | 61.29kD |
| NCBI Full Name: | L-2-hydroxyglutarate dehydrogenase, mitochondrial |
| NCBI Synonym Full Names: | L-2-hydroxyglutarate dehydrogenase |
| NCBI Official Symbol: | L2HGDH |
| NCBI Official Synonym Symbols: | L2HGA; C14orf160 |
| NCBI Protein Information: | L-2-hydroxyglutarate dehydrogenase, mitochondrial |
| UniProt Protein Name: | L-2-hydroxyglutarate dehydrogenase, mitochondrial |
| UniProt Synonym Protein Names: | Duranin |
| Protein Family: | L-2-hydroxyglutarate dehydrogenase |
| UniProt Gene Name: | L2HGDH |
 | Western blot analysis of extracts of various cell lines, using L2HGDH antibody (CAB7996) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s. |
 | Immunohistochemistry of paraffin-embedded human stomach using L2HGDH antibody (CAB7996) at dilution of 1:100 (40x lens). |
 | Immunohistochemistry of paraffin-embedded mouse heart using L2HGDH antibody (CAB7996) at dilution of 1:100 (40x lens). |
| Williams et al. | Signalling metabolite L-2-hydroxyglutarate activates the transcription factor HIF-1α in lipopolysaccharide-activated macrophages | J Biol Chem.2021 | PubMed ID: 34929172 |
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