PEO1 Antibody (ARP88216_P050)
Product Specifications
CAS Number
9007-83-4
Gene Name
Twinkle mtDNA helicase
Gene Aliases
PEO, PEO1, SCA8, ATXN8, IOSCA, PEOA3, SANDO, TWINL, MTDPS7, PRLTS5, C10orf2
Gene ID
56652
Swiss Prot
Q96RR1
Accession Number
NP_001157284.1
Reactivity
Human
Immunogen
The immunogen is a synthetic peptide directed towards the C terminal region of human PEO1
Target
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.
Clonality
Polyclonal
Type
Polyclonal Antibody
Sequence
KARLKKIKDDTGPVAKKPSSGKKGATTQNSEICSGQAPTPDQPDTSKRSK
Applications
WB
Purification
Affinity purified
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions
Reconstitution & Storage Instructions
Western Blotting/Immunoblotting (WB/IB) Protocol
Western Blotting/Immunoblotting (WB/IB) Protocol
Immunohistochemistry (IHC) Protocol
Immunohistochemistry (IHC) Protocol
Immunocytochemistry (ICC) Protocol
Immunocytochemistry (ICC) Protocol
Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol
Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol
Blocking Peptide Competition Protocol (BPCP)
Blocking Peptide Competition Protocol (BPCP)
Immunoprecipitation (IP) Protocol
Immunoprecipitation (IP) Protocol
Antibody Array (AA) Protocol
Antibody Array (AA) Protocol
Concentration
0.5 mg/ml
Format
Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Reconstitution
For short term use, store at 2-8C up to 1 week. For long term storage, store at -20C in small aliquots to prevent freeze-thaw cycles.
Molecular Weight
77 kDa
Protein Length
684
NCBI Gene Symbol
TWNK
Host or Source
Rabbit
Protein Name
Twinkle protein, mitochondrial
Gene Name URL
TWNK
Nucleotide Accession Number
NM_001163812.1
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