Describe supplementation of end products as an approach for chronic management of metabolic disorders, and provide a specific example.
What will be an ideal response?
Substrates that should have been formed from the enzymatic reactions that are defective in metabolic disorders are needed for energy production, protein turnover, feedback inhibition of metabolic pathways, and/or production of other substrates. In the case of metabolic blocks in these enzymatic reactions, an alternative means of achieving the end goal of the reaction must be provided. In glycogen storage disease type I, for example, the enzymatic block results in an inability to produce glucose. Consequently, providing a continuous source of glucose through frequent feedings is the therapy for this disorder. Similarly, in the amino acid disorder homocystinuria, where the amino acid homocysteine is unable to be converted to cysteine, cysteine consequently needs to be supplemented in the diet.
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The best dietary source of vitamin K is __________.
a. brown rice b. raw kale c. potatoes d. collard greens
Now that you have learned more about the relationship between blood lipids and health, you might want to think more about James, the little boy who was featured in this chapter’s Everybody Has a Story piece. Why was James’s physician so concerned about his elevated blood cholesterol? How could this be harmful to his health?
In developing nations with high rates of malnutrition, the main type of food available is:
a. green and root vegetables. b. cereals and grains. c. milk and eggs. d. surplus commodities.
Which of the following nutrients is not required by law to be added to refined grain products?
a. riboflavin b. folate c. magnesium d. thiamin e. niacin