This gene encodes a hexameric DNA helicase which unwinds short stretches of double-stranded DNA in the 5' to 3' direction and, along with mitochondrial single-stranded DNA binding protein and mtDNA polymerase gamma, is thought to play a key role in mtDNA replication.
The protein localizes to the mitochondrial matrix and mitochondrial nucleoids.
Mutations in this gene cause infantile onset spinocerebellar ataxia (IOSCA) and progressive external ophthalmoplegia (PEO) and are also associated with several mitochondrial depletion syndromes.
Alternative splicing results in multiple transcript variants encoding distinct isoforms.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of C10orf2. Species Crossreactivity: Human, mouse, rat
Isotype：IgG
Calc Applications Abbrev：IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Recombinant full length Human C10orf2.
Shelf Life：1year
Swiss Prot Number：Q96RR1
EU Commodity Code：30021010
商品名：C10orf2 (Ataxin 8, Ataxin8, ATXN 8, ATXN8, C10 orf2, C10orf 2, C10orf2, Chromosome 10 Open Reading Frame 2, IOSCA, Mitochondrial, MTDPS7, PEO 1, PEO, PEO1, PEO1_HUMAN, PEOA3, Progressive external ophthalmoplegia 1 Protein, SANDO, SCA 8, SCA8, T7 gp4 like Protein with intraMitochondrial nucleoid localization, T7 gp4-like Protein with intraMitochondrial nucleoid localization, T7 Helicase-Related Protein with intraMitochondrial nucleoid localization, T7 like Mitochondrial DNA Helicase, T7-like Mitochondrial DNA Helicase, Twinkle Protein, Twinkle Protein, Mitochondrial, TWINL,) 100ul
