the fun side of biochemistry

Carbs?? Disease.

This weeks lecture was based on Carbohydrates.. One major type of carbohydrate is GLYCOGEN.. Glycogen is a polysaccharide that is the storage form of glucose in animals(humans). After eating, there is too much glucose in the blood. our bodies response to this is to store the excess glucose as glycogen in the liver and muscle cells.

Glycogen storage diseases are genetic enzyme deficiencies. It results in excessive glycogen accumulation within cells. Additional symptoms depend on the particular enzyme that is deficient.

There are 11 forms of Glycogen Storage Diseases (aka Glycogenoses). Some are the Type I GSD or Von Gierke’s disease, caused by hepatic deficiency of Glucose 6 Phosphatase, the Type II GSD or Pompe’s disease, caused by deficiency of acid alpha-glucosidase, the Type IV or Andersen’s Disease, caused by deficit of branching enzyme in various organs, including the liver, and the GSD Type V or McArdle’s Disease, caused by muscle deficiency of Glycogen Phosphorylase, among others.Image

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