TWNK Antilichaam - middelste regio : HRP (ARP36482_P050-HRP)

Twinkle mtDNA helicase

PEO, PEO1, SCA8, ATXN8, IOSCA, PEOA3, SANDO, TWINL, MTDPS7, PRLTS5, C10orf2

56652

Q96RR1

NP_068602

Human, Mouse, Rat, Cow, Guinea Pig, Horse

The immunogen is a synthetic peptide directed towards the middle region of human PEO1

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.

ZBTB1; BMI1; SMAD9; ICT1; SQSTM1; AKTIP

HRP: Horseradish Peroxidase

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 86%; Guinea Pig: 79%; Horse: 79%; Human: 100%; Mouse: 86%; Rat: 86%

Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.

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.

77kDa

Farge, G., (2008) Nucleic Acids Res. 36 (2), 393-403

Wet Ice

684

TWNK

TWNK

Rabbit

Twinkle protein, mitochondrial

NM_021830

GDVGVFPLEFNKNSLTFSIPPKNKARLKKIKDDTGPVAKKPSSGKKGATT