C10orf2 Antibody
Product Specifications
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
Curated Selection
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