Overview

  • Product name

    Recombinant Human PDHB protein
  • Protein length

    Protein fragment

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Species

      Human
    • Sequence

      LEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQF GVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDI IFAIKKTLNI
    • Amino acids

      250 to 359
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab152601 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 mg/ml.

     This product was previously labelled as Pyruvate Dehydrogenase E1 beta subunit

     

  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • DKFZp564K0164
    • mitochondrial
    • ODPB_HUMAN
    • pdhB
    • PDHBD
    • PDHE1 B
    • PDHE1-B
    • PHE1B
    • Pyruvate dehydrogenase (lipoamide) beta
    • Pyruvate dehydrogenase E1 beta polypeptide
    • Pyruvate dehydrogenase E1 component subunit beta
    • Pyruvate dehydrogenase E1 component subunit beta mitochondrial
    see all
  • Function

    The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3).
  • Involvement in disease

    Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).
  • Cellular localization

    Mitochondrion matrix.
  • Information by UniProt

Images

  • ab152601 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab152601 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab152601.
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