The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
Chromosome 10 open reading frame 2
Progressive external ophthalmoplegia 1 protein
T7 gp4 like protein with intramitochondrial nucleoid localization
T7 gp4-like protein with intramitochondrial nucleoid localization
T7 helicase-related protein with intramitochondrial nucleoid localization
T7 like mitochondrial DNA helicase
T7-like mitochondrial DNA helicase
Twinkle protein, mitochondrial
Involved in mitochondrial DNA (mtDNA) metabolism. Could function as an adenine nucleotide-dependent DNA helicase. Function inferred to be critical for lifetime maintenance of mtDNA integrity. In vitro, forms in combination with POLG, a processive replication machinery, which can use double-stranded DNA (dsDNA) as template to synthesize single-stranded DNA (ssDNA) molecules. May be a key regulator of mtDNA copy number in mammals.
High relative levels in skeletal muscle, testis and pancreas. Lower levels of expression in the heart, brain, placenta, lung, liver, kidney, spleen, thymus, prostate, ovary, small intestine, colon and leukocytes. Expression is coregulated with MRPL43.
Involvement in disease
Progressive external ophthalmoplegia with mitochondrial DNA deletions, autosomal dominant, 3 Sensory ataxic neuropathy dysarthria and ophthalmoparesis Mitochondrial DNA depletion syndrome 7
Contains 1 SF4 helicase domain.
Mitochondrion matrix > mitochondrion nucleoid. Colocalizes with mtDNA in mitochondrial nucleoids, a nucleoproteins complex consisting of a number of copies of proteins associated with mtDNA, probably involved in mtDNA maintenance and expression.