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Mitochondrial genome maintenance exonuclease

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Metal-dependent single-stranded DNA (ssDNA) exonuclease involved in mitochondrial genome maintenance. Has preference for 5'-3' exonuclease activity but is also capable of endoduclease activity on linear substrates. Necessary for maintenance of proper 7S DNA levels. Probably involved in mitochondrial DNA (mtDNA) repair, possibly via the processing of displaced DNA containing Okazaki fragments during RNA-primed DNA synthesis on the lagging strand or via processing of DNA flaps during long-patch base excision repair. Specifically binds 5-hydroxymethylcytosine (5hmC)-containing DNA in stem cells.

Below are the list of possible Mitochondrial genome maintenance exonuclease products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Mitochondrial genome maintenance exonuclease 1

 Mitochondrial genome maintenance exonuclease 1 ELISA Kit
 Mitochondrial genome maintenance exonuclease 1 Recombinant
 Mitochondrial genome maintenance exonuclease 1 Antibody
MGME1: an endo-/exonuclease involved in mitochondrial genome maintenance. Lacks detectable ribonuclease activity. Degrades DNA mainly in a 3'5' direction with a strong preference for single-stranded DNA. Requires free ends for its activity. Does not degrade circular substrates. Probably involved in mitochondrial DNA (mtDNA) repair, possibly via the processing of displaced DNA containing Okazaki fragments during RNA-primed DNA synthesis on the lagging strand or via processing of DNA flaps during long- patch base excision repair. Overexpression decreases the levels of 7S DNA, suggesting an impor
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tant role in 7S DNA regulation. Defects in this gene are a cause of progressive external ophthalmoplegia with mitochondrial DNA deletions (PEOAX), characterized by external ophthalmoplegia, emaciation and respiratory failure. Muscle biopsies show mtDNA depletion and multiple mtDNA deletions. A protein of the PD-(D/E)XK superfamily and MGME1 family. Belongs to the MGME1 family.

Protein type: Deoxyribonuclease; EC 3.1.-.-; Mitochondrial

Chromosomal Location of Human Ortholog: 20p11.23

Cellular Component: mitochondrion

Molecular Function: single-stranded DNA specific exodeoxyribonuclease activity

Biological Process: mitochondrial DNA repair; mitochondrial DNA replication; mitochondrial genome maintenance

Disease: Mitochondrial Dna Depletion Syndrome 11
 MGME1 ELISA Kit
 MGME1 Recombinant
 MGME1 Antibody
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 C20orf72 Recombinant
 C20orf72 Antibody
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 DDK1 Recombinant
 DDK1 Antibody
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