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Paper Information

Journal:   IRANIAN JOURNAL OF CHILD NEUROLOGY (IJCN)   FALL 2012 , Volume 6 , Number 4 (SUPPL 1); Page(s) 1 To 2.
 
Paper: 

LYSOSOMAL STORAGE DISEASE

 
 
Author(s):  GHOFRANI MOHAMMAD*
 
* MOFID CHILDREN HOSPITAL, SHARIATI AVE, TEHRAN, IRAN
 
Abstract: 

Lysosomes are cytoplasmic organelles containing hydrolytic enzymes that degrade the macromolecules proteins of cellular catabolism. The causes of lysosomal enzyme disorder stem from
1. Impaired enzyme synthesis
2. Abnormal enzyme targeting
3. Defect of structure of Accessory factor which is needed for enzymes function. Clinical manifestations depend on the organ (s) involved.

 
Keyword(s): LYSOSOME, ENZYME, GENE THERAPY, CHILD
 
References: 
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