Anti-L2HGDH antibody (ab230194)
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Overview
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Product name
Anti-L2HGDH antibody
See all L2HGDH primary antibodies -
Description
Rabbit polyclonal to L2HGDH -
Host species
Rabbit -
Tested applications
Suitable for: IHC-Pmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Orangutan -
Immunogen
Recombinant fragment corresponding to Human L2HGDH aa 1-120.
Sequence:MVPALRYLVGACGRARGLFAGGSPGACGFASGRPRPLCGGSRSASTSSFD IVIVGGGIVGLASARALILRHPSLSIGVLEKEKDLAVHQTGHNSGVIHSG IYYKPESLKAKLCVQGAALLYEYCQQKGISYKQCGKLIVAVEQEEIPRLQ ALYEKGLQNGVPGLRLIQQEDIKKKEPYCRGLMAIDCPHTGIVDYRQVAL SFAQDFQEAGGSVLTNFEVK
Database link: Q9H9P8 -
Positive control
- IHC-P: Human testis tissue.
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.3
Preservative: 0.02% Sodium azide
Constituents: PBS, 50% Glycerol -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
Our Abpromise guarantee covers the use of ab230194 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application | Abreviews | Notes |
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IHC-P | 1/20 - 1/200. |
Target
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Tissue specificity
Widely expressed. Highly expressed in brain, testis and muscle. Expressed to a lower extent in lymphocytes, fibroblasts, keratinocytes, placenta, bladder, small intestine, liver and bone marrow. -
Involvement in disease
Defects in L2HGDH are the cause of L-2-hydroxyglutaric aciduria (L2HGA) [MIM:236792]. 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. -
Sequence similarities
Belongs to the L2HGDH family. -
Cellular localization
Mitochondrion. - Information by UniProt
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Database links
- Entrez Gene: 79944 Human
- Entrez Gene: 100172827 Orangutan
- Omim: 609584 Human
- SwissProt: Q9H9P8 Human
- SwissProt: Q5R9N7 Orangutan
- Unigene: 256034 Human
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Alternative names
- 2 hydroxyglutarate dehydrogenase antibody
- Alpha hydroxyglutarate oxidoreductase antibody
- Alpha ketoglutarate reductase antibody
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Images
Datasheets and documents
References
ab230194 has not yet been referenced specifically in any publications.