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Two types of mitochondrial crystals in diseased human skeletal muscle fibers
Author(s) -
Farrants George W.,
Fil Sven Hovmöller,
Stadhouders Ad. M.
Publication year - 1988
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880110109
Subject(s) - crystallography , type (biology) , crystal (programming language) , skeletal muscle , mitochondrial intermembrane space , biophysics , chemistry , anatomy , materials science , biology , bacterial outer membrane , biochemistry , gene , ecology , escherichia coli , computer science , programming language
Abstract Mitochondrial crystalline inclusions, frequently found in mitochondrial myopathies, were analyzed by crystallographic techniques and computer‐aided image processing. It could be shown that these structures were real crystals. There are two distinct types of crystal, which can be distinguished by shape, size, and pattern. So‐called type I crystals are usually present in the intracristal space, whereas the type II crystals are preferentially located in the intermembrane space between outer and inner mitochondrial membranes. The unit cell dimensions were found to be 38 × 34 × 8 nm for the type I crystals and 20 × 17 × 8 nm for the type II crystals. These results strongly suggest that the crystals are composed of macromolecules, presumably proteins. Arguments are presented that indicate that type I crystals occur only in type 1 muscle fibers and type II crystals in type 2 muscle fibers.