Get the facts on Glycogen Storage Disease Type III treatment, diagnosis, staging, causes, types, symptoms. Information and current news about clinical trials and trial-related data, Glycogen Storage Disease Type III prevention, screening, research, statistics and other Glycogen Storage Disease Type III related topics. We answer all your qestions about Glycogen Storage Disease Type III.
Question: my bf has glycogen storage disease type iii..can anyone help with info about it? He said it causes him to store less energy so he can't do such activities as play sports for to long etc. In all seriousness, I am a little worried about things like can it pass on to our kids? How will he perform sexually? Any help would be great..and if you're a doctor or you have a doctor family member you can ask, it would be even better :) I really appreciate it! thank you!
Answer: Use the links and read the reports.
Question: Glycogen Storage Disease. 10 points!! =]]? In terms of glycogen, how is Type II and Type III of GSD similar.
In terms of organelles involved, how is Type I GSD different from type II.
Thank you so much! I really appreciate anyones help. I will give the ten points right away.
Answer: (Q1) In terms of glycogen, how is Type II and Type III of GSD similar?
(A1) Types II and III both fail to adequately catabolize glycogen, causing excessive glycogen accumulation in the liver, muscles, and (sometimes) the heart.
(Q2) In terms of organelles involved, how is Type I GSD different from type II?
(A2) The defective organelle in Type I is the endoplasmic reticulum. The defective organelle in Type II is the lysosome.
GSD type I (known as Von Gierke's disease) is caused by a glucose-6-phosphatase deficiency, and is the most common of the glycogen storage diseases. Glucose-6-phosphatase is an enzyme located on the inner membrane of the endoplasmic reticulum. The catalytic unit is associated with a calcium binding protein, and three transport proteins (T1, T2, T3) that facilitate movement of glucose-6-phosphate (G6P), glucose, and phosphate (respectively) into and out of the enzyme. This deficiency impairs the ability of the liver to produce free glucose from glycogen and from gluconeogenesis, causing severe hypoglycemia. Reduced glycogen breakdown results in increased glycogen storage in liver and kidneys, causing enlargement of both. Other metabolic derangements include lactic acidosis and hyperlipidemia.
GSD type II (Pompe's disease) is the result of an acid maltase deficiency. It is the only glycogen storage disease with a defect in lysosomal metabolism, and was the first glycogen storage disease to be identified, in 1932. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and nervous system.
GSD type III (aka. Cori's disease, Forbe's disease, or limit dextrinosis) is a consequence of a glycogen debrancher enzyme (i.e., amylo-1,6-glucosidase) deficiency. This causes excess amounts of an abnormal glycogen to be deposited in the liver, muscles and, in some cases, the heart.
