COQ8A Polyclonal Antibody
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-8071R |
| Product Name | COQ8A Polyclonal Antibody |
| Applications |
WB
ELISA
IHC-P
IHC-F
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Reactivity | Human, Rat |
| Predicted Reactivity | Mouse, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human ADCK3/CABC1 |
| Immunogen Range | 501-647/647 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Shipped at 4℃. Store at -20℃ for one year. 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 |
| ELISA | 1:500-1000 |
| IHC-P | 1:200-400 |
| IHC-F | 1:100-500 |
| 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 (bs-8071R) validated for, and at what dilution?
bs-8071R is validated for WB (1:300-5000); ELISA (1:500-1000); IHC-P (1:200-400); IHC-F (1:100-500); 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 (bs-8071R) 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 be stored?
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. Storage buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise COQ8A Polyclonal Antibody (bs-8071R)?
KLH conjugated synthetic peptide derived from human ADCK3/CABC1 Immunogen range: 501-647/647.