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Overview

Properties

Applications

Our Abpromise guarantee covers the use of ab57541 in the following tested applications.

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

Application Notes
WB
  • Application notesWB: Use at a concentration of 1-5 µg/ml.


    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.

    • Target

    • FunctionMay be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.
    • Tissue specificityUbiquitously expressed with a relatively greater abundance in heart and skeletal muscle.
    • Involvement in diseaseDefects in ADCK3 are a cause of coenzyme Q10 deficiency (CoQ10 deficiency) [MIM:607426]. CoQ10 deficiency is an autosomal recessive disorder with variable manifestations. It can be associated with three main clinical phenotypes: a predominantly myopathic form with central nervous system involvement, an infantile encephalomyopathy with renal dysfunction and an ataxic form with cerebellar atrophy.
      Defects in ADCK3 are the cause of spinocerebellar ataxia autosomal recessive type 9 (SCAR9) [MIM:612016]; also known as autosomal recessive cerebellar ataxia type 2 (ARCA2). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR9 is an autosomal recessive form characterized by gait ataxia and cerebellar atrophy with slow progression and few associated features. Patients can manifest brisk tendon reflexes and Hoffmann sign, mild psychomotor retardation, mild axonal degeneration of the sural nerve, exercise intolerance and elevated serum lactate.
    • Sequence similaritiesBelongs to the protein kinase superfamily. ADCK protein kinase family.
      Contains 1 protein kinase domain.
    • Cellular localizationMitochondrion.

      • Target information above from: UniProt accession Q8NI60 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt
    • Database links
      • Alternative names
          aarF domain containing protein kinase 3 antibodyaarF domain-containing protein kinase 3 antibodyADCK 3 antibody
          Adck3 antibodyADCK3_HUMAN antibodyCABC 1 antibodyCABC1 antibodyChaperone ABC1 (activity of bc1 complex S.pombe) like antibodyChaperone ABC1 activity of bc1 complex homolog antibodyChaperone ABC1 like antibodyChaperone activity of bc1 complex like antibodyChaperone activity of bc1 complex like mitochondrial antibodyChaperone activity of bc1 complex-like antibodyChaperone-ABC1-like antibodyCoenzyme Q8 homolog antibodyCOQ 8 antibodyCOQ8 antibodyMGC4849 antibodymitochondrial antibody
        see all

      Anti-ADCK3 antibody images

      • Western blot - CABC1 antibody (ab57541)
        Western blot - CABC1 antibody (ab57541)


        Predicted band size : 72 kDa
        CABC1 antibody (ab57541) at 1ug/lane + HeLa cell lysate at 25ug/lane.

      References for Anti-ADCK3 antibody (ab57541)

      ab57541 has not yet been referenced specifically in any publications.

      Publishing research using ab57541 ? Please let us know so that we can cite the reference in this datasheet

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