What makes carbamoyl phosphate

Abstract Carbamoyl phosphate synthetase catalyzes the production of carbamoyl phosphate from bicarbonate, glutamine, and two molecules of MgATP. Cited By. This article is cited by publications.

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Eilers , Alexander B. Alleman , Burak V. Kabasakal , Jennifer N. Wells , James W. Murray , Boguslaw P. Nocek , Jennifer L. In this condition, the carbamoyl phosphate synthetase I enzyme is at low levels deficient or absent, and the urea cycle cannot proceed normally.

As a result, nitrogen accumulates in the bloodstream in the form of toxic ammonia instead of being converted to less toxic urea and excreted. Ammonia is especially damaging to the brain, and excess ammonia causes neurological problems and other signs and symptoms of carbamoyl phosphate synthetase I deficiency. This condition is inherited in an autosomal recessive pattern , which means both copies of the gene in each cell have mutations.

The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition. Genetics Home Reference has merged with MedlinePlus. Learn more. The information on this site should not be used as a substitute for professional medical care or advice.

Contact a health care provider if you have questions about your health. Carbamoyl phosphate synthetase I deficiency. From Genetics Home Reference. Description Carbamoyl phosphate synthetase I deficiency is an inherited disorder that causes ammonia to accumulate in the blood hyperammonemia. Frequency Carbamoyl phosphate synthetase I deficiency is a rare disorder; its overall incidence is unknown. Inheritance This condition is inherited in an autosomal recessive pattern , which means both copies of the gene in each cell have mutations.

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