Early-onset liver mtDNA depletion and late-onset proteinuric nephropathy in Mpv17 knockout mice.

In addition, a significant trend toward reduction in glomerular mtDNA content was already detected in non-proteinuric, 6-month-old Mpv17 −/− mice, implicating that proteinuria is the result of a chronic pathogenic process ultimately damaging glomerular structure and function.

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