C10ORF2
Reactivity: Human, Mouse, Rat
WB
Host: Rabbit
Polyclonal
unconjugated
Application Notes
Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Format
Liquid
Concentration
0.25 mg/mL
Buffer
PBS containing 0.09 % (W/V) sodium azide as preservative
Preservative
Sodium azide
Precaution of Use
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice
Avoid repeated freezing and thawing.
Storage
4 °C/-20 °C
Storage Comment
Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.
Target
C10orf2 (C10ORF2)
(Chromosome 10 Open Reading Frame 2 (C10ORF2))
Alternative Name
PEO1 / Twinkle
Background
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.Synonyms: C10orf2, Progressive external ophthalmoplegia 1 protein, T7-like mitochondrial DNA helicase, Twinkle protein mitochondrial