Recombinant Human Pyruvate Dehydrogenase E1 beta subunit protein (ab152601)

Overview

  • Product name
    Recombinant Human Pyruvate Dehydrogenase E1 beta subunit 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
      proprietary 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.
  • 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

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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