C10orf2 Antibody

• 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.

• NCBI Gene ID:

  56652

• Swiss Prot:

  Q96RR1

• Accession Number:

  Q96RR1

• Host:

  Rabbit

• Reactivity:

  Human

• Immunogen:

  This C10orf2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 656-684 amino acids from the C-terminal region of human C10orf2.

• Clonality:

  Polyclonal

• Conjugation:

  Unconjugated

• Type:

  Primary Antibodies

• Field of Research:

  Neuroscience

• Purification:

  This antibody is purified through a protein A column, followed by peptide affinity purification.

• Concentration:

  Batch dependent

• Buffer:

  Supplied in PBS with 0.09% (W/V) sodium azide.

• Modification:

  None

• Shipping Conditions:

  Blue Ice

• Storage Conditions:

  Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

• Calculated Molecular Weight:

  77 kDa

• Fragment:

  Rabbit Ig

• Symbol:

  PEO1

• NCBI Official Name:

  Twinkle protein, mitochondrial

• NCBI Organism:

  Homo sapiens

• Background Reference 01:

  Longley, M.J., et al. J. Biol. Chem. 285 (39) :29690-29702 (2010)

• Background Reference 02:

  Wang, W., et al. Nucleic Acids Res. (2010) In press :

• Background Reference 03:

  Fratter, C., et al. Neurology 74 (20) :1619-1626 (2010)

• Background Reference 04:

  Kruger, J., et al. Mol Neurodegener 5, (2010) :

• Other Product Names:

  Twinkle protein, mitochondrial, Progressive external ophthalmoplegia 1 protein, T7 gp4-like protein with intramitochondrial nucleoid localization, T7-like mitochondrial DNA helicase, PEO1, C10orf2

• Tested Applications:

  WB, IHC-P

• Protein ID:

  74752111

• Physical Properties:

  Liquid