I. The Problem

The three diseases in this study are borne out of errors that involve certain components of the Krebs Cycle. These conditions have a wide array of manifestations that can be seen as early as infancy. Since this pathway is central to energy production, it can affect most types of tissue, with most of the symptoms observed in nerve and muscle tissues. (Rustin et al., 1997)

II. Guide Question
1. What are the three major pathways that eventually become entry points of molecules into Krebs Cycle? (5 pts)
2. How is a “committed step” defined in the context of a metabolic pathway and why are they
important? Which steps and/or enzymes are involved in the committed steps in the Krebs
Cycle? What are the possible implications of these steps were deregulated? (10 pts)
3. What are the three Krebs Cycle inborn errors mentioned in the paper? Why are they called
deficiencies? What do you think are the probable causes for these? (10 pts)
4. In your own words, what do you think is/are the reason/s why most of the clinical features
of these diseases involve muscle and nerve tissue? (10 points)
5. Given clinical manifestations, what suggestion do you have pertaining to management of the diseases mentioned? (5 pts)