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Long name
CABC1 Primary Polyclonal Antibody
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Also known as
CABC1 PAb
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Category
Primary Antibodies
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Conjugation
Unconjugated
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Target Antigen
CABC1
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Specificity
This is a highly specific antibody against CABC1.
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Modification s
None
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Modification site s
Unmodified antibody
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Clonality
Polyclonal antibody
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Clone number
Polyclonal antibody
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Concentration
1ug per 1ul
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Source
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human ADCK3/CABC1
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Tested Applications
WB, IHC-P, IF(IHC-P)
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Recommended dilutions
WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
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Cross reactivity
Human, Mouse, Rat
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Cross reactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Background of the target antigen
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.
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Purification method
This antibody was purified via Protein A.
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Storage conditions
Keep the antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at -20°C for up to 1 year.
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Synonym names
mitochondrial; aarF domain containing protein kinase 3; aarF domain-containing protein kinase 3; ADCK 3; ADCK3; ADCK3_HUMAN; CABC 1; 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; COQ8.
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Properties
If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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French translation
anticorps
