Description

  • Product name

    Recombinant Human SDHA protein (His tag)
    See all SDHA proteins and peptides
  • Purity

    > 90 % SDS-PAGE.

  • Expression system

    Escherichia coli
  • Accession

  • Protein length

    Protein fragment
  • Animal free

    No
  • Nature

    Recombinant
    • Species

      Human
    • Sequence

      SAKVSDSISAQYPVVDHEFDAVVVGAGGAGLRAAFGLSEAGFNTACVTKL FPTRSHTVAAQGGINAALGNMEEDNWRWHFYDTVKGSDWLGDQDAIHYMT EQAPAAVVELENYGMPFSRTEDGKIYQRAFGGQSLKFGKGGQAHRCCCVA DRTGHSLLHTLYGRSLRYDTSYFVEYFALDLLMENGECRGVIALCIEDGS IHRIRAKNTVVATGGYGRTYFSCTSAHTSTGDGTAMITRAGLPCQDLEFV QFHPTGIYGAGCLITEGCRGEGGILINSQGERFMERYAPVAKDLASRDVV SRSMTLEIREGRGCGPEKDHVYLQLHHLPPEQLATRLPGISETAMIFAGV DVTKEPIPVLPTVHYNMGGIPTNYKGQVLRHVNGQDQIVPGLYACGEAAC ASVHGANRLGANSLLDLVVFGRACALSIEESCRPGDKVPPIKPNAGEESV MNLDKLRFADGSIRTSELRLSMQKSMQNHAAVFRVGSVLQEGCGKISKLY GDLKHLKTFDRGMVWNTDLVETLELQNLMLCALQTIYGAEARKESRGAHA REDYKVRIDEYDYSKPIQGQQKKPFEEHWRKHTLSYVDVGTGKVTLEYRP VIDKTLNEADCATVPPAIRSY
    • Predicted molecular weight

      84 kDa including tags
    • Amino acids

      44 to 664
    • Tags

      His tag N-Terminus
    • Additional sequence information

      N-terminal 6xHis-SUMO tag.

Specifications

Our Abpromise guarantee covers the use of ab226453 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

  • Form

    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    Constituents: Tris buffer, 50% Glycerol

General Info

  • Alternative names

    • CMD1GG
    • DHSA_HUMAN
    • Flavoprotein subunit of complex II
    • Fp
    • PGL5
    • SDH 2
    • SDH1
    • SDH2
    • SDHA
    • SDHF
    • Succinate dehydrogenase [ubiquinone] flavoprotein subunit
    • Succinate dehydrogenase [ubiquinone] flavoprotein subunit mitochondrial
    • Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
    • Succinate dehydrogenase complex flavoprotein subunit
    • Succinate dehydrogenase complex flavoprotein subunit A
    • Succinate dehydrogenase complex flavoprotein subunit precursor
    • Succinate dehydrogenase complex subunit A
    • Succinate dehydrogenase complex subunit A flavoprotein
    • Succinate dehydrogenase complex subunit A flavoprotein (Fp)
    see all
  • Function

    Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q).
  • Pathway

    Carbohydrate metabolism; tricarboxylic acid cycle; fumarate from succinate (eukaryal route): step 1/1.
  • Involvement in disease

    Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D) [MIM:252011]. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome.
    Defects in SDHA are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
    Defects in SDHA are the cause of cardiomyopathy dilated type 1GG (CMD1GG) [MIM:613642]. CMD1GG is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
  • Sequence similarities

    Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily.
  • Cellular localization

    Mitochondrion inner membrane.
  • Information by UniProt

Images

  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

References

ab226453 has not yet been referenced specifically in any publications.

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