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. 2022 Feb;33(2):305–325. doi: 10.1681/ASN.2021050596

Figure 4.

Figure 4.

Renal biopsies of two MT-TF patients show abnormal mitochondria in distal parts of the nephron. (A–C) Transmission electron microscopy of the renal biopsy sample of patient 3.III.1. (D and E) Transmission electron microscopy of a percutaneous renal biopsy sample of patient 10.II.3. (A) Representative image of a perpendicular cross-section of the distal tubule, with a large number of abnormally shaped and sized mitochondria (two examples indicated with white arrows). Cristae profiles appear distorted, including some mitochondria with no discernable cristae. Nanotunneling visible (three examples indicated with white arrowheads). Magnification, ×1000. (B) Close-up of atypical giant mitochondrion of >3 µm in length (same as indicated by the left arrow in panel [A]). Note the large size and compartmentalization. Magnification, ×6000. (C) Close-up of atypical mitochondria (not in panel [A]). Note the concentric cristae (onion-like appearance). Magnification, ×6000. (D) Representative image of a perpendicular cross-section of the distal tubule; enlarged mitochondria are visible. (E) A close-up of panel (D) shows an almost complete lack of cristae structure in most mitochondria.