Final answer:
The two urea cycle enzymes located inside the mitochondria are Ornithine Transcarbamylase and Carbamoyl Phosphate Synthetase I, which are essential for the conversion of ammonia to urea.
Step-by-step explanation:
The two urea cycle enzymes that are located inside the mitochondria are Ornithine Transcarbamylase (OTC) and Carbamoyl Phosphate Synthetase I (CPS-I). Ornithine Transcarbamylase catalyzes the reaction where ornithine and carbamoyl phosphate combine to form citrulline, which later gets transported out of the mitochondria. Carbamoyl Phosphate Synthetase I is the enzyme responsible for the formation of carbamoyl phosphate from ammonium ion, bicarbonate ion, and ATP, which is the very first step of the urea cycle.
The first two steps of the urea cycle occur in the mitochondria, and these involve the enzymes Ornithine Transcarbamylase (OTC) and Carbamoyl Phosphate Synthetase I (CPS-I), while the following reactions take place in the cytoplasm. Deficiency in any of these enzymes, particularly Ornithine Transcarbamylase, can lead to an accumulation of toxic levels of ammonia in the body.