/*! This file is auto-generated */ .wp-block-button__link{color:#fff;background-color:#32373c;border-radius:9999px;box-shadow:none;text-decoration:none;padding:calc(.667em + 2px) calc(1.333em + 2px);font-size:1.125em}.wp-block-file__button{background:#32373c;color:#fff;text-decoration:none} Problem 8 A deficiency of a citric acid cy... [FREE SOLUTION] | 91Ó°ÊÓ

91Ó°ÊÓ

A deficiency of a citric acid cycle enzyme in both mitochondria and the cytosol of some tissues (e.g., blood lymphocytes) results in severe neurological abnormalities in newborns. The disease is characterized by excretion in the urine of abnormally large amounts of \(\alpha\)-ketoglutarate, succinate, and fumarate. What enzyme deficiency would lead to these symptoms?

Short Answer

Expert verified
The enzyme deficiency that would lead to these symptoms is fumarase.

Step by step solution

01

Understanding the Krebs Cycle

The Krebs cycle is a series of enzymatic reactions that catalyze the aerobic metabolism of fuel molecules to carbon dioxide and water, thereby generating energy for the production of ATP molecules. \(\alpha\)-ketoglutarate, succinate, and fumarate are intermediate products of this cycle. Therefore, their excess in the urine suggests an interruption or malfunction in this cycle.
02

Identify the Enzyme

Based on the information given, the specific enzyme must be involved in the conversion of \(\alpha\)-ketoglutarate to succinate and succinate to fumarate. Therefore, we have to look at the steps of the cycle which transform \(\alpha\)-ketoglutarate to succinate and then to fumarate. The enzyme involved after \(\alpha\)-ketoglutarate in the cycle is the \(\alpha\)-ketoglutarate dehydrogenase complex. This enzyme catalyzes the conversion of \(\alpha\)-ketoglutarate into succinyl-CoA. Then, succinyl-CoA is converted into succinate by succinyl-CoA synthetase. Lastly, succinate is converted into fumarate by the enzyme succinate dehydrogenase. Given that the excretion of all three, \(\alpha\)-ketoglutarate, succinate, and fumarate is increased, these compounds are not being converted into their subsequent metabolites within the cycle, meaning that the enzymatic dysfunction occurs after them in the cycle.
03

Conclusion

Hence, the enzyme that fits this description is fumarate hydratase, also known as fumarase. Fumarase is the enzyme that catalyzes the conversion of fumarate, the last mentioned compound in the problem, into malate within the Krebs cycle. This means that since fumarase is deficient, fumarate cannot be converted into malate and is instead excreted through urine, causing the symptoms mentioned in the problem.

Unlock Step-by-Step Solutions & Ace Your Exams!

  • Full Textbook Solutions

    Get detailed explanations and key concepts

  • Unlimited Al creation

    Al flashcards, explanations, exams and more...

  • Ads-free access

    To over 500 millions flashcards

  • Money-back guarantee

    We refund you if you fail your exam.

Over 30 million students worldwide already upgrade their learning with 91Ó°ÊÓ!

One App. One Place for Learning.

All the tools & learning materials you need for study success - in one app.

Get started for free

Most popular questions from this chapter

The disease beriberi, which results from a dietary deficiency of vitamin \(\mathrm{B}_{1}\) (thiamine), is characterized by neurologic and cardiac symptoms, as well as increased levels of pyruvate and \(\alpha\)-ketoglutarate in the blood. How does a deficiency of thiamine account for the increased levels of pyruvate and \(\alpha\)-ketoglutarate?

Patients in shock experience decreased delivery of \(\mathrm{O}_{2}\) to tissues, decreased activity of the pyruvate dehydrogenase complex, and increased anaerobic metabolism. Excess pyruvate is converted to lactate, which accumulates in tissues and in the blood, causing lactic acidosis. (a) Since \(\mathrm{O}_{2}\) is not a reactant or product of the citric acid cycle, why do low levels of \(\mathrm{O}_{2}\) decrease the activity of the pyruvate dehydrogenase complex? (b) To alleviate lactic acidosis, shock patients are sometimes given dichloroacetate, which inhibits pyruvate dehydrogenase kinase. How does this treatment affect the activity of the pyruvate dehydrogenase complex?

The activities of PFK-2 and fructose 2,6-bisphosphatase are contained in a bifunctional protein that effects tight control over glycolysis and gluconeogenesis through the action of fructose 2,6-bisphosphate. Describe another protein that contains kinase and phosphatase activities in a single protein molecule. What pathways does it control?

(a) The citric acid cycle converts one molecule of citrate to one molecule of oxaloacetate, which is required for the cycle to continue. If other cycle intermediates are depleted by being used as precursors for amino acid biosynthesis, can a net synthesis of oxaloacetate occur from acetyl CoA via the enzymes of the citric acid cycle? (b) How can the cycle continue to function if insufficient oxaloacetate is present?

Pyruvate dehydrogenase complex deficiency is a disease that results in various metabolic and neurological effects. Pyruvate dehydrogenase complex deficiency can cause lactic acidosis in affected children. Other clinical symptoms include increased concentrations of pyruvate and alanine in the blood. Explain the increase in the levels of pyruvate, lactate, and alanine in individuals with pyruvate dehydrogenase complex deficiency.

See all solutions

Recommended explanations on Chemistry Textbooks

View all explanations

What do you think about this solution?

We value your feedback to improve our textbook solutions.

Study anywhere. Anytime. Across all devices.