The glycogen storage disease is the abnormal storage of glycogen in the body tissues. Glucose being major source of energy; it is stored in the form of glycogen and later released in the form of glucose with the help of enzymes, the process known as gluconeogenesis. Glycogen is a complex material composed of glucose molecules linked together in long chains with many branches off the chains. Glycogen is found mainly in liver and muscle cells, while the kidneys and intestines are minor storage sites.
In Glycogen Storage Disease (GSD) there is inherited absence or deficiency of any of the enzymes responsible for glycogenesis or glycogenolysis, needed by the body during exercise or between meals. These enzyme defects lead to abnormal tissue concentrations of glycogen or to structurally abnormal forms of glycogen. The enzyme may be important in all cells of the body, or cells found specifically in liver or muscle tissue. Thus, the many forms of GSD can be described as affecting primarily the liver, the muscles, or both.
In all types of GSD, also called as glycogenosis, the body is not able to produce sufficient glucose in the blood stream or to utilize glucose as a source of energy. Diagnosis of the type of GSD is made on the basis of symptoms, physical examination and biochemical tests. A muscle or liver biopsy confirms the actual enzyme defect.
There are about eleven known types of GSD, which are classified by a number, by the name of the defective enzyme, or by the name of the author who first described the condition.
Almost all forms of GSD occur when a child inherits the affected gene.
Dr. Rajneesh Kumar Sharma
Homoeo Cure & Research Centre P. Ltd.
NH 74, Moradabad Road, Kashipur (Uttaranchal) 244713 India
Ph- 05947- 275535, 260327, 274338, 277418
Fax- 274338, 275535; Cells- 98 976 18594, 98 976 21896
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Sub-editorGlycogen Storage Disease :: Information on Glycogen storage disease
by Sub-editor ( Author at Spirit India )
Posted on November 9th, 2005 at 12:44 am.
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