Monday 30 December 2013

Introduction


Most people start their day with breakfast, while others might opt for brunch instead. Regardless of whether it is breakfast or brunch, the food serves as a form of energy for us to perform our task. Do you know that glucose found in food is a main source of energy for us? Glucose is a form of carbohydrate and has an important role in metabolism. Now, you will start wondering what human metabolism is. Do not fret. Metabolism simply refers to the breakdown of food and its subsequent transformation into energy to meet a person's energy demands at the cellular level.

Let us begin the story of glucose, using cereal as our example. Firstly, the cereals that you consumed during breakfast will be broken down by enzymes into simple sugars and other products. Simple sugars like glucose are rapidly absorbed into the bloodstream, and this raises your blood sugar. The higher your blood sugar rises after meals and the longer the blood sugar remains high, the more likely you are to develop diabetic complications. Thus, to prevent this from happening, our body will store this excess glucose in the bloodstream as glycogen in the liver and muscles. When the blood glucose level in the body drops below a normal range, the glycogen will be converted back to glucose by enzymes.

Now imagine if one enzyme involved so far, was defective. It would result in either improperly formed glycogen, or glycogen would not get broken down into glucose. This person will suffer from a type of Glycogen Storage Disease (GSD). So what are GSDs? It is a group of inherited genetic disorders that causes glycogen to be incorrectly formed or released in the body. Glycogen is mainly stored in the liver or muscle tissues. As a result, GSDs usually have an effect on the liver, muscles, or both. 

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