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ProteomicsMice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria
Replication of mammalian mitochondrial DNA (mtDNA) is an essential process that requires high fidelity and control at multiple levels to ensure proper mitochondrial function. Mutations in the mitochondrial genome maintenance exonuclease 1 (MGME1) gene were recently reported in mitochondrial disease ...
ORGANISM(S): Homo sapiens (Human) 
2018-07-11 | PXD009138 | Pride
Mtdna deletions Mgme1 knock out Progeria 
ProteomicsSubcellular fraction kidney proteomics in MGME1 knockout mice
Metabolic dysregulation can lead to inflammatory responses. Imbalanced nucleotide synthesis triggers the release of mitochondrial DNA (mtDNA) to the cytosol and an innate immune response by cGAS-STING signaling. However, how nucleotide deficiency drives mtDNA dependent inflammation has not been eluc...
ORGANISM(S): Mus musculus (Mouse) 
2025-07-25 | PXD053639 | Pride
Mgme1 Mitochondria Kidney 
UnknownMice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria.
Not available
S-EPMC5865154 | biostudies-literature
UnknownMice lacking the mitochondrial exonuclease MGME1 develop inflammatory kidney disease with glomerular dysfunction.
Not available
S-EPMC9119528 | biostudies-literature
UnknownStructural basis of how MGME1 processes DNA 5' ends to maintain mitochondrial genome integrity.
Not available
S-EPMC11040146 | biostudies-literature
UnknownLinear mtDNA fragments and unusual mtDNA rearrangements associated with pathological deficiency of MGME1 exonuclease.
Not available
S-EPMC4222359 | biostudies-literature
UnknownMGME1 associates with poor prognosis and is vital for cell proliferation in lower-grade glioma.
Not available
S-EPMC10449294 | biostudies-literature
UnknownStructural insights into DNA degradation by human mitochondrial nuclease MGME1.
Not available
S-EPMC6237815 | biostudies-literature
UnknownAbsence of both MGME1 and POLG EXO abolishes mtDNA whereas absence of either creates unique mtDNA duplications.
Not available
S-EPMC11002302 | biostudies-literature
UnknownHomozygous c.359del variant in MGME1 is associated with early onset cerebellar ataxia.
Not available
S-EPMC6379073 | biostudies-literature
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