Overview

  • Product name

  • Description

    Rabbit polyclonal to L2HGDH
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Mouse, Human
    Predicted to work with: Orangutan
  • Immunogen

    Recombinant fragment corresponding to Human L2HGDH aa 1-220.
    Sequence:

    MVPALRYLVGACGRARGLFAGGSPGACGFASGRPRPLCGGSRSASTSSFD IVIVGGGIVGLASARALILRHPSLSIGVLEKEKDLAVHQTGHNSGVIHSG IYYKPESLKAKLCVQGAALLYEYCQQKGISYKQCGKLIVAVEQEEIPRLQ ALYEKGLQNGVPGLRLIQQEDIKKKEPYCRGLMAIDCPHTGIVDYRQVAL SFAQDFQEAGGSVLTNFEVK


    Database link: Q9H9P8

  • Positive control

    • WB: Mouse heart lysate. IHC-P: Human testis tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab230230 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
WB 1/1000 - 1/5000. Detects a band of approximately 50 kDa (predicted molecular weight: 50 kDa).
IHC-P 1/20 - 1/200.

Target

  • 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
  • Database links

  • Alternative names

    • 2 hydroxyglutarate dehydrogenase antibody
    • Alpha hydroxyglutarate oxidoreductase antibody
    • Alpha ketoglutarate reductase antibody
    • C14orf160 antibody
    • Duranin antibody
    • FLJ12618 antibody
    • L alpha hydroxyglutarate dehydrogenase antibody
    • L-2-hydroxyglutarate dehydrogenase antibody
    • L-2-hydroxyglutarate dehydrogenase, mitochondrial antibody
    • L2HDH_HUMAN antibody
    • l2hgdh antibody
    • mitochondrial antibody
    see all

Images

  • Anti-L2HGDH antibody (ab230230) at 1/1000 dilution + Mouse heart tissue lysate

    Secondary
    Goat polyclonal to rabbit IgG at 1/10000 dilution

    Predicted band size: 50 kDa
    Observed band size: 50 kDa

  • Paraffin-embedded human testis tissue stained for L2HGDH using ab230230 at 1/100 dilution in immunohistochemical analysis.

References

ab230230 has not yet been referenced specifically in any publications.

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