MABN814MI
MilliporeSigma™ Mouse anti-Twinkle, Clone: 13A10.1,
Manufacturer: MilliporeSigma™
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | MABN814MI-Each-of-1 | In Stock | ₹ 38,958.86 |
MABN814MI - Each of 1
In Stock
Quantity
1
Base Price: ₹ 38,958.86
GST (18%): ₹ 7,012.595
Total Price: ₹ 45,971.455
Antigen
Twinkle
Classification
Monoclonal
Formulation
Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1M Tris-Glycine (pH 7.4), 150mM NaCl with 0.05% sodium azide.
Gene Symbols
EPO1; C10orf2
Immunogen
GST-tagged recombinant protein corresponding to the C-terminal of human Twinkle.
Quantity
100 μg
Research Discipline
Neuroscience
Gene ID (Entrez)
NP_068602
Content And Storage
Stable for one year at 2°C to 8°C from date of receipt.
Isotype
IgG2a κ
Applications
Immunohistochemistry (Paraffin), Western Blot
Clone
13A10.1
Gene Accession No.
Q96RR1
Host Species
Mouse
Purification Method
Protein G Purified
Regulatory Status
RUO
Primary or Secondary
Primary
Target Species
Human
Form
Purified
Related Products
Description
- 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.