| Host: | Rabbit |
| Applications: | WB/ELISA |
| Reactivity: | Mouse |
| Note: | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Clonality : | Polyclonal |
| Conjugation: | Unconjugated |
| Isotype: | IgG |
| Formulation: | PBS with 0.01% Thimerosal, 50% Glycerol, pH 7.3. |
| Purification: | Affinity purification |
| Concentration: | Lot specific |
| Dilution Range: | WB:1:500-1:1000 ELISA:Recommended starting concentration is 1 Mu g/mL. Please optimize the concentration based on your specific assay requirements. |
| Storage Instruction: | Store at-20°C for up to 1 year from the date of receipt, and avoid repeat freeze-thaw cycles. |
| Gene Symbol: | TWNK |
| Gene ID: | 56652 |
| Uniprot ID: | PEO1_HUMAN |
| Immunogen Region: | 585-684 |
| Specificity: | A synthetic peptide corresponding to a sequence within amino acids 585-684 of human C10orf2 (NP_068602.2). |
| Immunogen Sequence: | NVLILQDRKLVTGPGKRYLQ VSKNRFDGDVGVFPLEFNKN SLTFSIPPKNKARLKKIKDD TGPVAKKPSSGKKGATTQNS EICSGQAPTPDQPDTSKRSK |
| Tissue Specificity | 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. |
| Function | Isoform 1: Mitochondrial helicase involved in mtDNA replication and repair. Might have a role in mtDNA repair. Has DNA strand separation activity needed to form a processive replication fork for leading strand synthesis which is catalyzed by the formation of a replisome complex with POLG and mtSDB. Preferentially unwinds DNA substrates with pre-existing 5'-and 3'- single-stranded tails but is also active on a 5'- flap substrate. Can dissociate the invading strand of immobile or mobile D-loop DNA structures irrespective of the single strand polarity of the third strand. In addition to its DNA strand separation activity, also has DNA strand annealing, DNA strand-exchange and DNA branch migration activities. Isoform 2: Lack DNA unwinding and ATP hydrolysis activities. Does not bind single-stranded or double-stranded DNA. |
| Protein Name | Twinkle Mtdna Helicase Progressive External Ophthalmoplegia 1 Protein T7 Gp4-Like Protein With Intramitochondrial Nucleoid Localization T7-Like Mitochondrial Dna Helicase Twinkle Protein - Mitochondrial |
| Database Links | Reactome: R-HSA-2151201 Reactome: R-HSA-9837999 |
| Cellular Localisation | Mitochondrion Matrix Mitochondrion Nucleoid Mitochondrion Inner Membrane Peripheral Membrane Protein 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 Associates With Phospholipid Membranes Via Electrostatic Binding Preferentially Associates With Membranes Enriched With Cardiolipin A Lipid Abundant In The Mitochondrial Inner Membrane Atpase And Helicase Activity Is Enhanced By Binding To Lipid Membranes |
| Alternative Antibody Names | Anti-Twinkle Mtdna Helicase antibody Anti-Progressive External Ophthalmoplegia 1 Protein antibody Anti-T7 Gp4-Like Protein With Intramitochondrial Nucleoid Localization antibody Anti-T7-Like Mitochondrial Dna Helicase antibody Anti-Twinkle Protein - Mitochondrial antibody Anti-TWNK antibody Anti-C10orf2 antibody Anti-PEO1 antibody |
Information sourced from Uniprot.org