Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MAARLLRGSLRVLGGHRAPRQLPAARCSHSGGEERLETPSAKKLTDIGIR RIFSPEHDIFRKSVRKFFQEEVIPHHSEWEKAGEVSREVWEKAGKQGLLG VNIAEHLGGIGGDLYSAAIVWEEQAYSNCSGPGFSIHSGIVMSYITNHGS EEQIKHFIPQMTAGKCIGAIAMTEPGAGSDLQGIKTNAKKDGSDWILNGS KVFISNGSLSDVVIVVAVTNHEAPSPAHGISLFLVENGMKGFIKGRKLHK MGLKAQDTAELFFEDIRLPASALLGEENKGFYYIMKELPQERLLIADVAI SASEFMFEETRNYVKQRKAFGKTVAHLQTVQHKLAELKTHICVTRAFVDN CLQLHEAKRLDSATACMAKYWASELQNSVAYDCVQLHGGWGYMWEYPIAK AYVDARVQPIYGGTNEIMKELIAREIVFDK
    • Molecular weight
      73 kDa including tags
    • Amino acids
      1 to 430

Associated products

Specifications

Our Abpromise guarantee covers the use of ab114591 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 µg/ul. Best use within three months from the date of receipt of this protein.
  • 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: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • ACAD4
    • ACADL
    • ACADL_HUMAN
    • Acyl Coenzyme A dehydrogenase long chain
    • Acyl-CoA dehydrogenase long chain
    • FLJ94052
    • LCAD
    • Long chain acyl CoA dehydrogenase
    • Long-chain specific acyl-CoA dehydrogenase, mitochondrial
    see all
  • Pathway
    Lipid metabolism; mitochondrial fatty acid beta-oxidation.
  • Involvement in disease
    Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM:201475]. An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting.
  • Sequence similarities
    Belongs to the acyl-CoA dehydrogenase family.
  • Cellular localization
    Mitochondrion matrix.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE showing ab114591 at approximately 73.41kDa stained with Coomassie Blue.

References

ab114591 has not yet been referenced specifically in any publications.

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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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