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Pyruvate Dehydrogenase E1-alpha subunit peptide (ab103930)

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Overview

  • Product namePyruvate Dehydrogenase E1-alpha subunit peptideSee all Pyruvate Dehydrogenase E1-alpha subunit proteins and peptides ...
  • Protein descriptionSynthetic peptide of Human Pyruvate Dehydrogenase E1-alpha subunit.(Note: the amino acid sequence is proprietary) This peptide was used as an immunogen for ab92695 - Pyruvate Dehydrogenase E1-alpha subunit antibody.
  • Protein length18 amino acids

    • Properties

  • Purity70 - 90% by HPLC
  • FormLiquid
  • Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • Storage bufferInformation available upon request.
  • Concentration information loading...
  • Research Areas

    • Protein info

    • Alternative names
        ODPA_HUMANPDHPDHA1
        PDHCE1APDHE1 A type IPDHE1-A type IPHE1APyruvate Dehydrogenase (lipoamide) alpha 1Pyruvate Dehydrogenase E1 alphaPyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
      see all
  • FunctionThe 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).
  • Tissue specificityUbiquitous.
  • Involvement in diseaseDefects in PDHA1 are a cause of pyruvate decarboxylase 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 (X-LS).
    Defects in PDHA1 are the cause of X-linked Leigh syndrome (X-LS) [MIM:308930]. X-LS is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes.
  • Cellular localizationMitochondrion matrix.

    • Target information above from: UniProt accession P08559 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt

    References for Pyruvate Dehydrogenase E1-alpha subunit peptide (ab103930)

    ab103930 has not yet been referenced specifically in any publications.

    Publishing research using ab103930 ? Please let us know so that we can cite the reference in this datasheet

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    Dear abcam technical support team: This customer raised an enquiry about ab103930 (Pyruvate Dehydrogenase E1-alpha subunit peptide), this customer wants to know the sequence of this peptide, or can you offer the amino acid range (ex. 200-300 amino acid)? ...

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    Thank you for contacting us.

    Ab103930 was used as the immunogen for ab92695 and falls within residues 200-300 of the Human Pyruvate Dehydrogenase E1-alpha subunit.

    Please let me know if you require any further assistance.

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