ADCK3 Antibody

Code CSB-PA001137
Size US$100
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  • Western Blot analysis of COLO205 cells using CABC1 Polyclonal Antibody
  • Western Blot analysis of A549 cells using CABC1 Polyclonal Antibody
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Product Details

Uniprot No.
Target Names
COQ8A
Alternative Names
aarF domain containing protein kinase 3 antibody; aarF domain-containing protein kinase 3 antibody; ADCK 3 antibody; Adck3 antibody; ADCK3_HUMAN antibody; ARCA2 antibody; CABC 1 antibody; CABC1 antibody; Chaperone ABC1 (activity of bc1 complex S.pombe) like antibody; Chaperone ABC1 activity of bc1 complex homolog antibody; Chaperone ABC1 like antibody; Chaperone activity of bc1 complex like antibody; Chaperone activity of bc1 complex like mitochondrial antibody; Chaperone activity of bc1 complex-like antibody; Chaperone-ABC1-like antibody; Coenzyme Q8 homolog antibody; COQ 8 antibody; COQ10D4 antibody; COQ8 antibody; COQ8; S. cerevisiae; homolog of antibody; MGC4849 antibody; mitochondrial antibody; OTTHUMP00000035734 antibody; OTTHUMP00000035737 antibody; SCAR9 antibody
Raised in
Rabbit
Species Reactivity
Human,Mouse,Rat
Immunogen
Synthesized peptide derived from the Internal region of Human CABC1.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, IHC, IF, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
IF 1:200-1:1000
ELISA 1:10000
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Function
Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: does not show any protein kinase activity. Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates. Shows an unusual selectivity for binding ADP over ATP.
Gene References into Functions
  1. Loss of COQ8a results in cerebellar ataxia and coenzyme Q deficiency. PMID: 27499294
  2. ADCK3/COQ8 localises to mitochondrial cristae and is targeted to this organelle via the presence of an N-terminal localisation signal PMID: 26866375
  3. work reveals Mg(2+)-dependent ATPase activity of ADCK3, providing strong support for the theoretical prediction of this protein being a functional atypical kinase. PMID: 25540914
  4. a structural foundation for investigating the role of transmembrane association in regulating the biological activity of ADCK3 PMID: 25216398
  5. Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis. PMID: 25498144
  6. These observations highlight the importance of screening for a potentially treatable cause, CABC1/ADCK3 mutations PMID: 22036850
  7. play an important role in mediating p53-inducible apoptosis through the mitochondrial pathway. PMID: 11888884
  8. CABC1 gene mutations in four ubiquinone-deficient patients in three distinct families were reported. PMID: 18319072
  9. Five additional mutations in ADCK3 were found in three patients with sporadic ataxia, including one known to have CoQ10 deficiency in muscle. PMID: 18319074

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Involvement in disease
Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
Subcellular Location
Mitochondrion. Membrane; Single-pass membrane protein.
Protein Families
Protein kinase superfamily, ADCK protein kinase family
Tissue Specificity
Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
Database Links

HGNC: 16812

OMIM: 606980

KEGG: hsa:56997

STRING: 9606.ENSP00000355739

UniGene: Hs.118241

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301-363-4651 (Available 9 a.m. to 5 p.m. CST from Monday to Friday)
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Address
7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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