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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

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