Did giant mitochondria delay muscle maturation? An uncommon congenital myopathy.

Abstract:

INTRODUCTION:Mitochondria are semi-autonomous organelles that are able to change their shape, size, location, and number inside the living cell. Mitochondrial division is an extremely important process, because cell survival depends on there being an adequate number of mitochondria in each cell. The dynamics of these organelles are particularly important for normal embryonic cell development. We studied morphological and ultrastructural features of muscle-cell immaturity, with the appearance of abnormal giant mitochondria, in a child with an unusual congenital myopathy. METHODS:The biceps brachii and quadriceps femoris muscles were analyzed at the light and electron microscopy levels. RESULTS:Ultrastructural features of muscle-cell immaturity were manifested in the presence of primary and mature myotubes coexisting with abnormal giant mitochondria. CONCLUSIONS:Failure of mitochondrial fusion/fission machinery at an early stage of development may lead to delayed muscle-cell maturation.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Fidziańska A,Glinka Z

doi

10.1002/mus.23365

subject

Has Abstract

pub_date

2012-07-01 00:00:00

pages

125-9

issue

1

eissn

0148-639X

issn

1097-4598

journal_volume

46

pub_type

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