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Millipore This Anti-Twinkle Antibody, Clone 13a10.1 Is Validated For Use In Western Blotting, Immunohistochemistry (Paraffin) For The Detection Of Twinkle. - Mill (Additional S&H or Hazmat Fees May Apply)
T7 gp4-like protein with intramitochondrial nucleoid localization
T7-like mitochondrial DNA helicase
Twinkle
Background Information
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.
Product Information
Format
Purified
Presentation
Purified mouse monoclonal IgG2aκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
Quality Level
MQ100
Applications
Application
This Anti-Twinkle Antibody, clone 13A10.1 is validated for use in Western Blotting, Immunohistochemistry (Paraffin) for the detection of Twinkle.
Key Applications
Western Blotting
Immunohistochemistry (Paraffin)
Application Notes
Western Blotting Analysis: 0.5 µg/mL from a representative lot detected Twinkle in 10 µg of K562 cell lysate. Immunohistochemistry Analysis: A 1:50 dilution from a representative lot detected Twinkle in human cerebral cortex and human skeletal muscle tissue.
Biological Information
Immunogen
GST-tagged recombinant protein corresponding to the C-terminal of human Twinkle.
Epitope
C-terminal
Clone
13A10.1
Concentration
Please refer to lot specific datasheet.
Host
Mouse
Isotype
IgG2aκ
Species Reactivity
Human
Antibody Type
Monoclonal Antibody
Entrez Gene Number
NP_068602
Gene Symbol
EPO1
C10orf2
Purification Method
Protein G Purified
UniProt Number
Q96RR1
Molecular Weight
~77 kDa observed
Product Usage Statements
Quality Assurance
Evaluated by Western Blotting in HL-60 cell lysate.
Western Blotting Analysis: 0.5 µg/mL of this antibody detected Twinkle in 10 µg of HL-60 cell lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
