Anti-ADCK3 antibody (ab221193)
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Overview
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Product name
Anti-ADCK3 antibody
See all ADCK3 primary antibodies -
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
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Form
Liquid -
Storage instructions
Shipped 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 buffer
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: PBS, 40% Glycerol -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
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 |
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ICC/IF | Use a concentration of 1 - 4 µg/ml. Fixation/Permeabilization: PFA/Triton X-100. |
Target
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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
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Database links
- Entrez Gene: 56997 Human
- Omim: 606980 Human
- SwissProt: Q8NI60 Human
- Unigene: 118241 Human
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Alternative names
- aarF domain containing protein kinase 3 antibody
- aarF domain-containing protein kinase 3 antibody
- ADCK 3 antibody
see all
Images
Datasheets and documents
References
ab221193 has not yet been referenced specifically in any publications.