Coenzyme Q8B (COQ8B) Antibody (Biotin)

169€ (20 µg)
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935106861
info@markelab.com
name
Coenzyme Q8B (COQ8B) Antibody (Biotin)
category
Primary Antibodies
provider
Abbexa
reference
abx316415
tested applications
ELISA
Description
ADCK4 Antibody (Biotin) is a Rabbit Polyclonal against ADCK4 conjugated to Biotin.
Documents del producto
Instrucciones
Data sheet
Product specifications
Category | Primary Antibodies |
Immunogen Target | Coenzyme Q8B (COQ8B) |
Host | Rabbit |
Reactivity | Human |
Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
Clonality | Polyclonal |
Conjugation | Biotin |
Isotype | IgG |
Purity | > 95% |
Purification | Purified by Protein G. |
Size 1 | 20 µg |
Size 2 | 50 µg |
Size 3 | 100 µg |
Size 4 | 200 µg |
Size 5 | 1 mg |
Form | Liquid |
Tested Applications | ELISA |
Buffer | 0.01 M PBS, pH 7.4, 0.03% Proclin-300 and 50% Glycerol. |
Availability | Shipped within 5-10 working days. |
Storage | Aliquot and store at -20°C. Avoid exposure to light. Avoid repeated freeze/thaw cycles. |
Dry Ice | No |
UniProt ID | Q96D53 |
Gene ID | 79934 |
NCBI Accession | NP_001136027.1, NM_001142555.2, NP_079152.3, NM_024876.3 |
OMIM | 615567 |
Alias | NPHS9,COQ8B |
Background | Antibody anti-ADCK4 |
Status | RUO |
Descripción
aarF domain-containing kinase 4 (ADCK4), also known as COQ8B, is a mitochondrial protein involved in the biosynthesis and regulation of coenzyme Q10 (CoQ10), an essential molecule in the mitochondrial electron transport chain for ATP production. ADCK4 is particularly important in kidney and podocyte function, and mutations in the ADCK4 gene are associated with CoQ10 deficiency-related nephrotic syndrome, which can lead to kidney dysfunction and proteinuria. Beyond its role in mitochondrial bioenergetics, ADCK4 is thought to stabilize CoQ biosynthetic complexes, ensuring efficient electron transport and reducing oxidative stress. ADCK4 dysfunction is linked to mitochondrial diseases, with CoQ10 supplementation often used as a therapeutic strategy to mitigate symptoms and restore energy metabolism.
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