Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MAAILGDTIMVAKGLVKLTQAAVETHLQHLGIGGELIMAARALQSTAVEQ IGMFLGKVQGQDKHEEYFAENFGGPEGEFHFSVPHAAGASTDFSSASAPD QSAPPSLGHAHSEGPAPAYVASGPFREAGFPGQASSPLGRANGRLFANPR DSFSAMGFQRRFFHQDQSPVGGLTAEDIEKARQAKARPENKQHKQTLSEH ARERKVPVTRIGRLANFGGLAVGLGFGALAEVAKKSLRSEDPSGKKAVLG SSPFLSEANAERIVRTLCKVRGAALKLGQMLSIQDDAFINPHLAKIFERV RQSADFMPLKQMMKTLNNDLGPNWRDKLEYFEERPFAAASIGQVHLARMK GGREVAMKIQYPGVAQSINSDVNNLMAVLNMSNMLPEGLFPEHLIDVLRR ELALECDYQREAACARKFRDLLKGHPFFYVPEIVDELCSPHVLTTELVSG FPLDQAEGLSQEIRNEICYNILVLCLRELFEFHFMQTDPNWSNFFYDPQQ HKVALLDFGATREYDRSFTDLYIQIIRAAADRDRETVRAKSIEMKFLTGY EVKVMEDAHLDAILILGEAFASDEPFDFGTQSTTEKIHNLIPVMLRHRLV PPPEETYSLHRKMGGSFLICSKLKARFPCKAMFEEAYSNYCKRQAQQ
    • Amino acids
      1 to 647
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab163460 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • 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: 0.31% Glutathione, 0.79% Tris HCl

General Info

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

Images

  • ab163460 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab163460 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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