Methylglyoxal-mediated Gpd1 activation restores the mitochondrial defects in a yeast model of mitochondrial DNA depletion syndrome

• Published in: Biochimica et biophysica acta. General subjects Vol. 1867; no. 5; p. 130328
• Main Authors: Mukherjee, Soumyajit, Das, Shubhojit, Bedi, Minakshi, Vadupu, Lavanya, Ball, Writoban Basu, Ghosh, Alok
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.05.2023
• Subjects: Animals; DNA, Mitochondrial - genetics; DNA, Mitochondrial - metabolism; Glycerol-3-Phosphate Dehydrogenase (NAD+) - metabolism; Glycerol-3-phosphate dehydrogenase 1; Humans; Mammals - metabolism; Membrane Proteins - metabolism; Methylglyoxal; Mitochondrial respiratory chain; Phospholipids; Pyruvaldehyde - metabolism; Saccharomyces cerevisiae - metabolism; Saccharomyces cerevisiae Proteins - genetics; Saccharomyces cerevisiae Proteins - metabolism; SYM1; Zebrafish - metabolism; MDDS; mtDNA; IMM; OXPHOS; Phospholipids; Mitochondrial respiratory chain; SYM1; Gut2; Glycerol-3-phosphate dehydrogenase 1; Methylglyoxal; PC; PE; PLs; Gpd2; Gpd1; MG; MRC

Human MPV17, an evolutionarily conserved mitochondrial inner-membrane channel protein, accounts for the tissue-specific mitochondrial DNA depletion syndrome. However, the precise molecular function of the MPV17 protein is still elusive. Previous studies showed that the mitochondrial morphology and c...