C10orf2 Antikörper (AA 39-635)

Produktnummer ABIN7874604

Der Kaninchen Polyklonal anti-C10orf2 Antikörper wird verwendet zum Nachweis von C10orf2 in Proben von Human, Maus und Ratte. Er wurde validiert für WB und ELISA.

Kurzübersicht für C10orf2 Antikörper (AA 39-635) (ABIN7874604)

Target: C10orf2 (C10ORF2) (Chromosome 10 Open Reading Frame 2 (C10ORF2))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser C10orf2 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-C10orf2 Antikörper

Bindungsspezifität: AA 39-635

Verwendungszweck: TWNK Antibody / Twinkle

Aufreinigung: Immunogen affinity purified

Immunogen: E.coli-derived human Twinkle/TWNK recombinant protein (Position: R39-K635) was used as the immunogen for the TWNK antibody.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the TWNK 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 TWNK 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 C10orf2

Target: C10orf2 (C10ORF2) (Chromosome 10 Open Reading Frame 2 (C10ORF2))

Andere Bezeichnung: TWNK

Hintergrund: TWNK antibody detects Twinkle, a mitochondrial DNA helicase essential for mtDNA replication and maintenance. Encoded by the TWNK gene on chromosome 10q24.31, this nuclear-encoded protein is imported into mitochondria, where it unwinds double-stranded DNA to facilitate replication of the mitochondrial genome. Twinkle is a member of the replicative helicase family related to bacteriophage T7 gene 4 helicase-primase, and it plays a vital role in mtDNA stability and copy number control.

Structurally, Twinkle forms a hexameric ring that encircles DNA and uses ATP hydrolysis to unwind duplex strands at replication forks. It cooperates with mitochondrial DNA polymerase gamma (POLG) and mitochondrial single-stranded DNA-binding protein (mtSSB) to sustain replication fidelity and genome integrity. Twinkle localizes to mitochondrial nucleoids, the structures where replication and transcription occur.

The TWNK antibody is widely used in mitochondrial biology, genetics, and metabolic research to study mtDNA replication, maintenance, and disorders of mitochondrial dysfunction. Western blot analysis detects a 77 kilodalton band corresponding to Twinkle, while immunofluorescence reveals mitochondrial localization with a punctate pattern. This antibody supports investigations into mechanisms of mtDNA propagation and repair.

Mutations in TWNK cause autosomal dominant progressive external ophthalmoplegia (PEO) and mitochondrial DNA depletion syndromes, leading to neuromuscular and metabolic disorders. Dysfunctional Twinkle results in replication stalling, deletions, and depletion of mitochondrial DNA. The TWNK antibody provides a reliable tool for studying mitochondrial replication machinery, genome stability, and related diseases. NSJ Bioreagents validates this antibody for western blotting, immunohistochemistry, and immunofluorescence, ensuring accurate detection for mitochondrial research.

UniProt: Q96RR1