Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.20
Preservative: 0.02% Sodium azide
Constituents: PBS, 40% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
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.
|ICC/IF||Use a concentration of 1 - 4 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100.
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.
- Information by UniProt
- aarF domain containing protein kinase 3 antibody
- aarF domain-containing protein kinase 3 antibody
- ADCK 3 antibody
ab221193 has not yet been referenced specifically in any publications.