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Spécifications
- Antibody type:Primary
- Antigen name:Chaperone activity of bc1 complex-like 1
- Clonality:Polyclonal
- Host:Rabbit
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Antigen symbol:CABC1
- Conjugation:Alexa Fluor® 555
- ImmunoChemistry:Yes
- Size:100 µL
- Cross adsorption:No
- Form:liquid
- Antigen synonyms:Chaperone activity of bc1 complex-like|ADCK 3|Chaperone activity of bc1 complex like mitochondrial|COQ 8|CABC 1|ADCK3|Chaperone ABC1 activity of bc1 complex homolog|Chaperone ABC1 activity of bc1 complex S.pombe like|aarF domain containing protein kinase 3|Chaperone activity of bc1 complex like|aarF domain-containing protein kinase 3|ADCK3_HUMAN|Coenzyme Q8 homolog|COQ8.|Chaperone ABC1 like|Chaperone-ABC1-like
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
- Storage temperature:Store at –20 °C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Purification:Purified by Protein A
- Cat. no.:10476-146
- Supplier No.:BS-8071R-A555
Spécifications
A propos de cet article
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). 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.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: CABC1
Clonality: Polyclonal
Clone:
Conjugation: Alexa Fluor® 555
Epitope:
Host: Rabbit
Isotype: IgG1
Reactivity: Human, Mouse, Rat