Discussion:
Fumarase Deficiency
Fumarase deficiency is an extremely rare disease in humans. People with this disease can survive although they are significantly affected.
a) Given the importance of fumarase to the citric acid cycle how is it possible for people with fumarase deficiency to produce adequate ATP for survival?
b) what would be the overall consequences of this deficiency for fuel catabolism in these people?
c) What would be the expected impact of this disease on the urea cycle?
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