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Merck
CN

MABN814

Anti-Twinkle Antibody, clone 13A10.1

clone 13A10.1, from mouse

Synonym(s):

Twinkle protein, mitochondrial, Progressive external ophthalmoplegia 1 protein, T7 gp4-like protein with intramitochondrial nucleoid localization, T7-like mitochondrial DNA helicase, Twinkle

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41
eCl@ss:
32160702
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Product Name

Anti-Twinkle Antibody, clone 13A10.1, clone 13A10.1, from mouse

isotype

IgG2aκ

biological source

mouse

conjugate

unconjugated

antibody form

purified immunoglobulin

antibody product type

primary antibodies

clone

13A10.1, monoclonal

species reactivity

human

technique(s)

immunohistochemistry: suitable (paraffin)
western blot: suitable

NCBI accession no.

UniProt accession no.

shipped in

wet ice

target post-translational modification

unmodified

Quality Level

Gene Information

human ... TWNK(56652)

General description

~77 kDa observed
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.

Analysis Note

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.

Application

This Anti-Twinkle Antibody, clone 13A10.1 is validated for use in Western Blotting, Immunohistochemistry (Paraffin) for the detection of Twinkle.
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.

Immunogen

GST-tagged recombinant protein corresponding to the C-terminal of human Twinkle.

Other Notes

Concentration: Please refer to lot specific datasheet.

Physical form

Format: Purified

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Storage Class

12 - Non Combustible Liquids

wgk

WGK 1

flash_point_f

Not applicable

flash_point_c

Not applicable


Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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