Human Recombinant ADCK3, Active protein, GST Tag (V2LY-0526-LY1946)

Name: Human Recombinant ADCK3, Active protein, GST Tag
SKU: V2LY-0526-LY1946
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Expressed Host

Baculovirus-Insect Cells

Protein Species

Human

Tag

GST Tag

Protein Construction

This product is Human Recombinant ADCK3, Active protein, GST Tag consist of Amino Acid: 156-end and predicts a molecular mass of 82 kDa.

Molecule Mass

82 kDa

Sequence

Amino Acid: 156-end

Species

Human

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity

Batch dependent.

Endotoxin

Please contact us for more information.

Format

Liquid

Buffer

Tirs, NaCl, Glycerol

Preservative

None

Storage

Store product at -70°C. For optimal storage, aliquot target into smaller quantities after centrifugation and store at recommended temperature. For most favorable performance, avoid repeated handling and multiple freeze/thaw cycles.

More Infomation

Target

Full Name

Coenzyme Q8A

Function

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25498144, PubMed:21296186, PubMed:25540914, PubMed:27499294).

Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:25498144, PubMed:27499294).

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 (PubMed:25498144, PubMed:27499294).

Shows an unusual selectivity for binding ADP over ATP (PubMed:25498144).

Biological Process

Phosphorylation Source: UniProtKB
Protein phosphorylation Source: GOC
Ubiquinone biosynthetic process Source: UniProtKB

Cellular Location

Mitochondrion; Membrane

Involvement in disease

Coenzyme Q10 deficiency, primary, 4 (COQ10D4):
An autosomal recessive disorder characterized by childhood-onset of cerebellar ataxia and exercise intolerance. Patient manifest gait ataxia and cerebellar atrophy with slow progression. Additional features include brisk tendon reflexes and Hoffmann sign, variable psychomotor retardation and variable seizures.

Topology

Helical: 214-230

Traschütz, A., Schirinzi, T., Laugwitz, L., Murray, N. H., Bingman, C. A., Reich, S., ... & Synofzik, M. (2020). Clinico‐genetic, imaging and molecular delineation of COQ8A‐ataxia: A multicenter study of 59 patients. Annals of neurology, 88(2), 251-263.

Galosi, S., Barca, E., Carrozzo, R., Schirinzi, T., Quinzii, C. M., Lieto, M., ... & Friedman, J. (2019). Dystonia-Ataxia with early handwriting deterioration in COQ8A mutation carriers: A case series and literature review. Parkinsonism & Related Disorders, 68, 8-16.

Jacobsen, J. C., Whitford, W., Swan, B., Taylor, J., Love, D. R., Hill, R., ... & Lehnert, K. (2017). Compound Heterozygous Inheritance of Mutations in Coenzyme Q8A Results in Autosomal Recessive Cerebellar Ataxia and Coenzyme Q 10 Deficiency in a Female Sib-Pair. In JIMD Reports, Volume 42 (pp. 31-36). Springer, Berlin, Heidelberg.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Alternative Versions

Human Recombinant ADCK3 (A337G, A339G), Active protein, GST Tag (CAT#: V2LY-0526-LY1944)

Human Recombinant ADCK3 (A339G), Active protein, GST Tag (CAT#: V2LY-0526-LY1945)

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Human Recombinant ADCK3 (A337G, A339G), Active protein, GST Tag (CAT#: V2LY-0526-LY1944)

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Human Recombinant ADCK3 (A339G), Active protein, GST Tag (CAT#: V2LY-0526-LY1945)

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Mouse Anti-COQ8A Recombinant Antibody (CBFYC-2101) (CAT#: V2LY-1206-LY798)

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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