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Paper Information

Journal:   IRANIAN JOURNAL OF CHILD NEUROLOGY (IJCN)   FALL 2012 , Volume 6 , Number 4 (SUPPL 1); Page(s) 11 To 11.
 
Paper: 

LYSOSOMAL MYOPATHIES

 
 
Author(s):  NILIPOUR YALDA*
 
* MOFID CHILDREN HOPSTIAL, SHARIATI AVE, TEHRAN, IRAN
 
Abstract: 

Lysosomal myopathies are hereditary myopathies characterized morphologically by the presence of autophagic vacuoles. Autophagy is an intracellular bulk degradation process, which is used by all cells to eliminate waste materials. Autophagy is considered to be essential for myocytes and the lysosomal system becomes prominent in certain muscle diseases.
In muscle pathology, lysosomal abnormalities are seen in three types of vacuoles: rimmed vacuoles which are most likely a secondarily induced lysosomal abnormality, autophagic vacuoles, which are usually large and contain glycogen, seen specifically in acid maltase deficiency and autophagic vacuoles with unique sarcolemmal features with acetyl cholinesterase activity (AVSF), which are seen in Danon disease and other related myopathies.

 
Keyword(s): LYSOSOMAL MYOPATHY, MYOPATHY, POMPE, ACID MALTASE DEFICIENCY, DANON
 
References: 
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