TY  - JOUR
T1  - Abnormal Myofibrillogenesis Potentiates Dystrophin Deficiency in Duchenne Muscular Dystrophy
AU - , Lawrence, M. Agius 
JO  - International Journal of Molecular Medicine and Advance Sciences
VL  - 2
IS  - 1
SP  - 76
EP  - 83
PY  - 2006
DA  - 2001/08/19
SN  - 1813-176x
DO  - ijmmas.2006.76.83
UR  - https://makhillpublications.co/view-article.php?doi=ijmmas.2006.76.83
KW  - Myofiber
KW  -hypertrophy
KW  -muscular
KW  -dystrophy
KW  -myofibrillogenesis
AB  - Myofiber hypertrophy occurs in Duchenne muscular dystrophy and appears to predispose specifically to necrosis of affected muscle cells.  Hypertrophy would constitute an abnormal response on the part of myofibers in muscular dystrophy that characterizes subsequent progression to necrosis.  Aberrant myofibrillogenesis would be implicated in such a process in a manner that would potentiate dystrophin deficiency as the individual myofibers undergo hypertrophy.   Abnormally induced gene transcription pathways would, in multiple ways, ultimately result in a complex setting that promotes sarcolemmal disruption.   Only in terms of such potentiation can one recognize a tendency for myofiber hypertrophy in the first instance and a subsequent evolution to individual myofiber necrosis.  Fibrosis develops as a series of injuries that are propagated by myofiber necrosis and loss, reflecting progressive and variable loss of dystrophin anchorage on sarcolemmae.
ER  -