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Tomogram of a cluster of fragmented mitochondria in a Barth syndrome lymphoblast. Barth syndrome (BTHS) is a rare X-linked disease characterized by dilated cardiomyopathy, skeletal myopathy, and neutropenia. The disease is associated with mutations of the TAZ gene, resulting in defective cardiolipin (CL), an important inner mitochondrial membrane component.

A: Slice of the tomogram with surface-rendered depiction of a 3-D model, showing the inner membrane in yellow, inner-membrane derived tubes in orange, and the outer membrane in blue. The boxes (0.4 mm x 0.4 mm) mark three segmented details. B, C, D: 3-D models of membranes in the corresponding boxes. The images were magnified and rotated to reveal details of interest. White arrowheads point to zones of inner membrane adhesion.

(Devrim Acehan)