COQ8A Polyclonal Antibody, APC Conjugated
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-8071R-APC-100uL |
| Product Name | COQ8A Polyclonal Antibody, APC Conjugated |
| Applications |
WB
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Reactivity | Human, Rat |
| Predicted Reactivity | Mouse, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit |
| Specifications | |
| Conjugation | APC |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human ADCK3/CABC1 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target | |
| Subcellular location | Cytoplasm |
| Synonyms | 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. |
| Background | 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. |
| Application Dilution | |
| WB | 1:300-5000 |
| IF(IHC-P) | 1:50-200 |
| IF(IHC-F) | 1:50-200 |
| IF(ICC) | 1:50-200 |
Frequently asked questions
Which applications is COQ8A Polyclonal Antibody, APC Conjugated (bs-8071R-APC) validated for, and at what dilution?
bs-8071R-APC is validated for WB (1:300-5000); IF(IHC-P) (1:50-200); IF(IHC-F) (1:50-200); IF(ICC) (1:50-200). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
Which species does COQ8A Polyclonal Antibody, APC Conjugated (bs-8071R-APC) react with?
Tested reactivity: Human, Rat. Predicted reactivity (expected from sequence homology but not yet tested): Mouse, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit.
How should bs-8071R-APC be stored?
Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. Storage buffer: Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise COQ8A Polyclonal Antibody, APC Conjugated (bs-8071R-APC)?
KLH conjugated synthetic peptide derived from human ADCK3/CABC1
What are the excitation and emission wavelengths of the APC conjugate (bs-8071R-APC)?
APC on bs-8071R-APC: excitation / emission 650nm/660nm.