Atypical Kinase COQ8A, Mitochondrial (ADCK3) Antibody

195€ (20 µl)
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935106861
info@markelab.com
name
Atypical Kinase COQ8A, Mitochondrial (ADCK3) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx006933
tested applications
ELISA, WB
Description
ADCK3 Antibody is a Rabbit Polyclonal Antibody against ADCK3.
Documents del producto
Instrucciones
Data sheet
Product specifications
Category | Primary Antibodies |
Immunogen Target | Atypical Kinase COQ8A, Mitochondrial (ADCK3) |
Host | Rabbit |
Reactivity | Human, Mouse, Rat |
Recommended Dilution | ELISA: 1 µg/ml, WB: 1/500 - 1/2000. Optimal dilutions/concentrations should be determined by the end user. |
Clonality | Polyclonal |
Conjugation | Unconjugated |
Isotype | IgG |
Purification | Purified by affinity chromatography. |
Size 1 | 20 µl |
Size 2 | 100 µl |
Size 3 | 2 × 100 µl |
Form | Liquid |
Tested Applications | ELISA, WB |
Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
Availability | Shipped within 5-10 working days. |
Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | Q8NI60 |
Gene ID | 56997 |
NCBI Accession | NP_064632.2 |
Alias | COQ8A,ARCA2,CABC1,COQ10D4,COQ8,SCAR9 |
Background | Antibody anti-ADCK3 |
Status | RUO |
Note | Concentration: > 0.2 mg/ml - |
Descripción
aarF domain-containing kinase 3 (ADCK3), also known as COQ8A, is a mitochondrial protein essential for coenzyme Q biosynthesis. It functions as a lipid kinase-like protein that stabilizes and regulates the synthesis of CoQ, which is crucial for electron transport and ATP production in mitochondria. Mutations in ADCK3 are linked to CoQ10 deficiency, which can result in a range of mitochondrial disorders, including cerebellar ataxia, seizures, and neuromuscular dysfunction. ADCK3 is highly expressed in energy-demanding tissues, including the brain and muscles, emphasizing its role in maintaining mitochondrial function and energy homeostasis. Therapeutic strategies for addressing ADCK3-related dysfunctions include CoQ10 supplementation and gene-targeted therapies, which aim to restore mitochondrial energy metabolism and reduce disease symptoms.
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