Renal Mitochondrial Cytopathies

Figure 3

Electron microscopy studies. Two examples of intense proliferation of abnormal mitochondria (swollen mitochondria with simplified or absent mitochondrial cristae) in a podocyte of a patient with a CoQ₁₀ biosynthesis defect (a) and in the proximal tubular cells of a child with severe De Toni-Debré-Fanconi syndrome (b).

(a)

(b)