人Pyruvate Dehydrogenase E1-alpha subunit肽(ab103930)

概述

描述

  • 性质Synthetic

技术指标

Our Abpromise guarantee covers the use of ab103930 in the following tested applications.

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

  • 纯度70 - 90% by HPLC.

  • 形式Liquid
  • 补充说明

    - First try to dissolve a small amount of peptide in either water or buffer. The more charged residues on a peptide, the more soluble it is in aqueous solutions.
    - If the peptide doesn’t dissolve try an organic solvent e.g. DMSO, then dilute using water or buffer.
    - Consider that any solvent used must be compatible with your assay. If a peptide does not dissolve and you need to recover it, lyophilise to remove the solvent.
    - Gentle warming and sonication can effectively aid peptide solubilisation. If the solution is cloudy or has gelled the peptide may be in suspension rather than solubilised.
    - Peptides containing cysteine are easily oxidised, so should be prepared in solution just prior to use.

  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Information available upon request.

常规信息

  • 别名
    • ODPA_HUMAN
    • PDH
    • PDHA
    • PDHA1
    • PDHCE1A
    • PDHE1 A type I
    • PDHE1-A type I
    • PHE1A
    • Pyruvate Dehydrogenase (lipoamide) alpha 1
    • Pyruvate dehydrogenase complex, E1 alpha polypeptide 1
    • Pyruvate Dehydrogenase E1 alpha
    • Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
    see all
  • 功能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).
  • 组织特异性Ubiquitous.
  • 疾病相关Defects 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.
  • 细胞定位Mitochondrion matrix.
  • Information by UniProt

Human Pyruvate Dehydrogenase E1-alpha subunit peptide (ab103930)参考文献

ab103930 has not yet been referenced specifically in any publications.

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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.

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