Overview

  • Product name

  • Description

    Rabbit polyclonal to ADCK3
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IFmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to Human ADCK3 aa 5-88.
    Sequence:

    LGDTIMVAKGLVKLTQAAVETHLQHLGIGGELIMAARALQSTAVEQIGMF LGKVQGQDKHEEYFAENFGGPEGEFHFSVPHAAG


    Database link: Q8NI60

  • Positive control

    • ICC/IF: HepG2 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab221193 in the following tested applications.

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

Application Abreviews Notes
ICC/IF Use a concentration of 1 - 4 µg/ml.

Fixation/Permeabilization: PFA/Triton X-100.

Target

  • Function

    May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.
  • Tissue specificity

    Ubiquitously expressed with a relatively greater abundance in heart and skeletal muscle.
  • Involvement in disease

    Defects 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 similarities

    Belongs to the protein kinase superfamily. ADCK protein kinase family.
    Contains 1 protein kinase domain.
  • Cellular localization

    Mitochondrion.
  • Information by UniProt
  • Database links

  • Alternative names

    • aarF domain containing protein kinase 3 antibody
    • aarF domain-containing protein kinase 3 antibody
    • ADCK 3 antibody
    • Adck3 antibody
    • ADCK3_HUMAN antibody
    • ARCA2 antibody
    • CABC 1 antibody
    • CABC1 antibody
    • Chaperone ABC1 (activity of bc1 complex S.pombe) like antibody
    • Chaperone ABC1 activity of bc1 complex homolog antibody
    • Chaperone ABC1 like antibody
    • Chaperone activity of bc1 complex like antibody
    • Chaperone activity of bc1 complex like mitochondrial antibody
    • Chaperone activity of bc1 complex-like antibody
    • Chaperone-ABC1-like antibody
    • Coenzyme Q8 homolog antibody
    • COQ 8 antibody
    • COQ10D4 antibody
    • COQ8 antibody
    • COQ8, S. cerevisiae, homolog of antibody
    • MGC4849 antibody
    • mitochondrial antibody
    • OTTHUMP00000035734 antibody
    • OTTHUMP00000035737 antibody
    • SCAR9 antibody
    see all

Images

  • Immunofluorescent analysis of PFA-fixed, Triton X-100 permeabilized HepG2 cells labeling ADCK3 with ab221193 at 4 µg/mL (green).

References

ab221193 has not yet been referenced specifically in any publications.

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