Abstract: Renal cells have a high density of mitochondria,resulting from the high energy demand for kidney to facilitate reabsorption of glomerular filtrate. Mutation of nuclear and mitochondrial DNA could both lead to mitochondrial cytopathies. Renal involvement in mitochondrial cytopathies associated with mitochondrial DNA mutation include tubular dysfunction (such as Fanconi syndrome and electrolyte disturbances), tubulointerstitial nephritis, and glomerular diseases (such as focal segmental glomerular sclerosis). And mitochondrial cytopathies could lead to endstage renal diseases eventually in a group of patients. The molecular mechanism of these cytopathies remain to be elucidated; Further research of mitochondrial nephropathy may provide important mechanistic insights and new therapeutic options.

Key words: mitochondrial DNA mutation, tubular dysfunction, tubulointerstitial nephritis, glomerular diseases