WBR0971

Author	[[PageAuthor::Rim Halaby, M.D. [1] (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Biochemistry
Sub Category	SubCategory::General Principles
Prompt	[[Prompt::In an experiment, mitonchondria are purified from sample of pig livers, and mitochondrial enzymes involved in the urea cycle are isolated. Fatty acylation assays are then carried out on the samples in order to study the regulatory role of fatty acylation in the kinetic activity of these enzymes. Which of the following enzymes is most likely present in the purified mitochondrial sample?]]
Answer A	AnswerA::Arginase
Answer A Explanation	[[AnswerAExp::This answer is incorrect because arginase is a cytoplasmic enzyme.]]
Answer B	AnswerB::Arginosuccinate synthetase
Answer B Explanation	[[AnswerBExp::This answer is incorrect because arginosuccinate synthetase is a cytoplasmic enzyme.]]
Answer C	AnswerC::Arginosuccinase
Answer C Explanation	[[AnswerCExp::This answer is incorrect because arginosuccinase is a cytoplasmic enzyme.]]
Answer D	AnswerD::Carbamoyl phosphate synthetase I
Answer D Explanation	[[AnswerDExp::Carbamoyl phosphate synthetase I is a mitochondrial enzyme involved in the urea cycle.]]
Answer E	AnswerE::Carbamoyl phosphate synthetase II
Answer E Explanation	[[AnswerEExp::This answer is incorrect because carbamoyl phosphate synthetase II is a cytoplasmic enzyme.]]
Right Answer	RightAnswer::C
Explanation	[[Explanation::The urea cycle involves a set of 5 hepatic enzymes that collectively convert ammonia into urea. A proportion of the reactions involved in urea synthesis occurs within the mitochondria, whereas the remaining reactions occur in the cytoplasm. The mitochondrial enzymes are: carbamoyl phosphate synthetase I and ornitithine transcarbamoylase (OTC). The cytoplasmic enzymes are: arginosuccinate synthetase, arginosuccinase and arginase. Shown below is a diagram depicting the different steps of the urea cycle. Educational objective: The mitochondrial enzymes involved in the urea cycle are carbamoyl phosphate synthetase I and ornitithine transcarbamoylase (OTC). Reference: M. Corv et al. Regulation of mitochondrial carbamoyl-phosphate synthetase 1 activity by active site fatty acylation. Journal of the biological chemistry (2001). First Aid for the USMLE step 1, 2013. Page 96-106. Educational Objective: References: ]]
Approved	Approved::No
Keyword	WBRKeyword::Urea cycle, WBRKeyword::Carbamoyl phosphate synthetase I
Linked Question	Linked::
Order in Linked Questions	LinkedOrder::