TWNK Antibody - C-terminal region : FITC (ARP75698_P050-FITC)
Selected Variant
ARP75698_P050-FITC
Size: 100 µL
Product Specifications
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_068602
Host
Rabbit
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.
Partner Proteins
ZBTB1; BMI1; SMAD9; ICT1; SQSTM1; AKTIP
Clonality
Polyclonal
Conjugation
FITC: Fluorescein Isothiocyanate
Type
Polyclonal Antibody
Applications
WB
Purification
Affinity purified
Concentration
0.5 mg/ml
Format
Liquid. Purified antibody supplied in 1x PBS buffer.
Reconstitution
All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.
Molecular Weight
75kDa
Shipping Conditions
Wet Ice
Protein Length
684
NCBI Gene Symbol
TWNK
NCBI GB Accession Number
TWNK
Host or Source
Rabbit
Protein Name
Twinkle protein, mitochondrial
Peptide Sequence
FRKFATDNNCHVTLVIHPRKEDDDKELQTASIFGSAKASQEADNVLILQD
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