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MilliporeSigma™ Mouse anti-Twinkle, Clone: 13A10.1,

Catalog No. MABN814MI Shop All MilliporeSigma Products
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MABN814MI 100 μg
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Description

Mouse Monoclonal Antibody

Specifically detects Twinkle clone: 13A10.1 in Human samples, and it is validated for Immunohistochemistry (Paraffin), Western Blotting

Twinkle protein, mitochondrial (EC 3.6.4.12; UniProt Q96RR1; also known as Ataxin 8, Mitochondrial twinkle protein, Progressive external ophthalmoplegia 1 protein, T7 gp4-like protein with intramitochondrial nucleoid localization, T7 helicase-related protein with intramitochondrial nucleoid localization, T7-like mitochondrial DNA helicase) is encoded by the EPO1 (also known as ATXN8, C10orf2, IOSCA, MTDPS7, PEO, PEO1, PEOA3, PRLTS5, SANDO, SCA8, TWINL) gene (Gene ID 56662) in human. Mitochondrial DNA (mtDNA) replication involves the catalytic and accessory subunits of DNA polymerase gamma, the mtDNA helicase Twinkle, the mitochondrial single-stranded DNA-binding protein, and the mitochondrial RNA polymerase. Cells expressing Twinkle mutants accumulate double-stranded mtDNA replication intermediates with loss of RNA associated with the lagging strand, consistent with the role of Twinkle in unwinding double-stranded mtDNA. Mutations in the human Twinkle gene (C10orf2) cause autosomal dominant progressive external ophthalmoplegia (PEO), associated with multiple mtDNA deletions.
TRUSTED_SUSTAINABILITY

Specifications

Antigen Twinkle
Applications Immunohistochemistry (Paraffin), Western Blot
Classification Monoclonal
Clone 13A10.1
Formulation Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% sodium azide.
Gene Accession No. Q96RR1
Gene Symbols EPO1; C10orf2
Host Species Mouse
Immunogen GST-tagged recombinant protein corresponding to the C-terminal of human Twinkle.
Purification Method Protein G Purified
Quantity 100 μg
Regulatory Status RUO
Research Discipline Neuroscience
Primary or Secondary Primary
Gene ID (Entrez) NP_068602
Target Species Human
Content And Storage Stable for one year at 2°C to 8°C from date of receipt.
Form Purified
Isotype IgG2a κ
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For Research Use Only

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