what exactly happen to glucose molecules when there is a deficincy or absence of lysosomal glucosidase?
how does it function in normal and ubnormal condition??
2007-03-16 06:09:13 · 3 answers · asked by pooh 1 in Science & Mathematics ➔ Medicine
The deficiency in lysosomal glucosidase in Pompe disease essentially does not allow breakdown of glycogen in the liver, in order to facilitate utilization of said glycogen stores during exercise or increased levels of activity in general. What then happens is accumulation of glycogen, because the lysosomes are not able to deconstruct the glycogen into glucose for use in the body.
manifest primarily as progressive muscle weakness because of insufficient energy available. Other manifestations include hepatomegaly (liver enlargement) due to excessive glycogen storage, heart enlargement, and neurological disorders, the latter 2 of which are very sensitive to glucose abnormalities.
2007-03-17 07:46:49 · answer #1 · answered by citizen insane 5 · 0⤊ 0⤋
Do you mean "Pompe's disease (Glycogen storage disease Type II)"?
Please see the web page on Glycogen storage diseases and Google search on and Pompe's disease.
2007-03-16 18:34:03 · answer #2 · answered by gangadharan nair 7 · 0⤊ 0⤋
don't know if it helps:
http://en.wikipedia.org/wiki/Pompe's_disease
2007-03-16 13:30:02 · answer #3 · answered by dave a 5 · 0⤊ 0⤋