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Ch. 5 - An Introduction to Carbohydrates
Chapter 5, Problem 9

Galactosemia is a potentially fatal disease that occurs in humans who lack the enzyme that converts galactose to glucose. If you were a physician treating a person with this disease, which of the following would you have them exclude from their diet? a. maltose b. starch c. mannose d. lactose

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Step 1: Understand the problem. The problem is asking which substance a person with galactosemia should avoid in their diet. Galactosemia is a condition where the body can't convert galactose to glucose due to the lack of a specific enzyme.
Step 2: Recall what each of the substances listed in the options are. Maltose is a sugar made from two glucose molecules, starch is a polysaccharide made from glucose, mannose is a sugar that can be converted to glucose, and lactose is a sugar made from glucose and galactose.
Step 3: Identify the substance that contains galactose. From the options given, only lactose contains galactose.
Step 4: Since people with galactosemia can't convert galactose to glucose, they should avoid substances that contain galactose.
Step 5: Therefore, the answer is lactose.

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Key Concepts

Here are the essential concepts you must grasp in order to answer the question correctly.

Galactosemia

Galactosemia is a genetic disorder characterized by the body's inability to metabolize galactose, a sugar found in milk and dairy products. This condition arises from a deficiency in the enzyme galactose-1-phosphate uridyltransferase, which is crucial for converting galactose into glucose. Without proper management, galactosemia can lead to serious health complications, including liver damage, cataracts, and intellectual disability.

Enzymatic Function

Enzymes are biological catalysts that facilitate biochemical reactions in the body. In the case of galactosemia, the lack of the enzyme responsible for converting galactose to glucose leads to the accumulation of galactose and its toxic metabolites. Understanding the role of enzymes in metabolic pathways is essential for recognizing how deficiencies can result in metabolic disorders and the dietary restrictions necessary to manage them.
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Dietary Management

Dietary management is a critical aspect of treating metabolic disorders like galactosemia. Patients are advised to avoid foods containing galactose, primarily found in dairy products and certain sugars. In this context, lactose, which is composed of glucose and galactose, must be excluded from the diet to prevent the harmful effects of galactose accumulation, highlighting the importance of dietary choices in managing health conditions.
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