hereditary defect in the body`s ability to metabolize glycogen, resulting in a muscle disorder that is usually fatal during the first year of life. ... [2 related articles]
Found on
http://www.britannica.com/eb/a-z/p/92
Synonym for type 2 glycogenosis ... Glycogenosis due to lysosomal alpha-1,4-glucosidase deficiency, resulting in accumulation of excessive amounts of glycogen of normal chemical structure in heart, muscle, liver, and nervous system. ... Synonym: generalised glycogenosis, Pompe's disease. ... (05 Mar 2000) ...
Found on
http://www.encyclo.co.uk/local/20973
Severe glycogen storage disease caused by deficiency in a (1-4)-glucosidase, the lysosomal enzyme responsible for glycogen hydrolysis. Even though the non-lysosomal glycogenolytic system is normal, glycogen still accumulates in the lysosomes.
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http://www.encyclo.co.uk/visitor-contributions.php
No exact match found.
