DKC1 Antikörper (AA 19-447)

Produktnummer ABIN7871593

Der Kaninchen Polyklonal anti-DKC1 Antikörper (ABIN7871593) detektiert spezifisch DKC1 in WB, IHC, IF, ELISA, ICC und FACS. Dieser Antikörper reagiert spezifisch mit Proben aus Human, Maus und Ratte.

Kurzübersicht für DKC1 Antikörper (AA 19-447) (ABIN7871593)

Target: DKC1 (Dyskeratosis Congenita 1, Dyskerin (DKC1))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser DKC1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Immunohistochemistry (IHC), Immunofluorescence (IF), ELISA, Immunocytochemistry (ICC), Flow Cytometry (FACS)

Produktdetails anti-DKC1 Antikörper

Bindungsspezifität: AA 19-447

Verwendungszweck: DKC1 Antibody / Dyskerin

Aufreinigung: Immunogen affinity purified

Immunogen: E.coli-derived human DKC1 recombinant protein (Position: R19-R447) was used as the immunogen for the DKC1 antibody.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the DKC1 antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: After reconstitution, the DKC1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Details zu DKC1

Target: DKC1 (Dyskeratosis Congenita 1, Dyskerin (DKC1))

Andere Bezeichnung: DKC1

Hintergrund: DKC1 antibody detects Dyskerin, a nucleolar pseudouridine synthase essential for ribosomal RNA modification, telomerase stability, and small nucleolar RNP biogenesis. The UniProt recommended name is Dyskerin (DKC1). This enzyme catalyzes the isomerization of uridine to pseudouridine in rRNA and snRNA, ensuring ribosome maturation and translation fidelity.

Functionally, DKC1 antibody identifies a 514-amino-acid protein localized in the nucleolus and Cajal bodies. DKC1 associates with NOP10, NHP2, and GAR1 to form the H/ACA ribonucleoprotein complex that modifies rRNA and snRNA. It also binds the telomerase RNA component (TERC), stabilizing the telomerase complex for telomere elongation.

The DKC1 gene is located on chromosome Xq28 and is ubiquitously expressed in dividing and metabolically active cells. Dyskerin function is vital for maintaining ribosome synthesis, RNA quality control, and telomere homeostasis. Its activity directly influences cell growth and chromosomal stability.

Pathologically, mutations in DKC1 cause X-linked dyskeratosis congenita, a rare disorder characterized by bone marrow failure, premature aging, and cancer predisposition. Reduced DKC1 function leads to defective rRNA modification, impaired telomerase activity, and genomic instability. Research using DKC1 antibody supports studies in RNA processing, telomere biology, and genetic diseases.

DKC1 antibody is validated for western blotting, immunofluorescence, and immunohistochemistry to detect pseudouridine synthases and nucleolar proteins. NSJ Bioreagents provides DKC1 antibody reagents optimized for studies in ribosome biogenesis, telomerase function, and RNA modification.

Structurally, Dyskerin features a TruB-like catalytic domain with conserved aspartate essential for pseudouridylation activity, and a nuclear localization signal directing nucleolar import. This antibody aids in elucidating DKC1's role in ribonucleoprotein assembly and RNA modification fidelity.

UniProt: O60832

Pathways: Telomere Maintenance