THE STRUCTURE, BIOSYNTHESIS AND DEGRADATION ASPECTS OF HUMAN GLYCOGEN: A REVIEW ON POTENTIAL GLYCOGEN STORAGE DISEASES AND THEIR TREATMENT

Areesha Batool*, Rida Fatima and Dr. Sikander Ali

ABSTRACT

This article deals with the introduction of glycogen, its native structure, general mechanism of metabolism, the defects associated with it and the possible remedies for the diseases. Glycogen-the polymeric carbohydrate is storage form of energy in most organisms. Glycogen is present in liver and skeletal muscles of humans. The proper synthesis and accurate degradation of the glycogen is necessary for maintenance of body functioning. Any defect in enzymes, that are involved in body metabolism, leads to diseases. Glycogen storage diseases are also the consequence of enzymatic disorders in glycogen metabolism. This article describes the different types of GSD depending upon the type of defected enzyme. Biochemical defects primarily glycogen storage diseases have now much more understanding and thus their remedies have been improved. So, patients are living much better and long lives. However, glycogen storage diseases are rare, and practitioner may only have few patients in a life time. Physician made a GSD differential by diagnostic test of enzymatic examination or by performing biopsies. Once the diagnosis is proven, suitable and proper treatments are performed. With marvelous progress in the field of molecular biology, techniques like gene therapy will be in use in the near future for such a challenging and perplexing group of metabolic disorders.

Keywords: Polymeric carbohydrates, Human glycogen, Glycogen metabolism, Glycogenosis, Defected enzymes, Possible treatment.