1. This gene belongs to the pyrroline -5-carboxylate reductase family. 2. The aroma is caused by the evaporation of 2-Acetyl-1-pyrroline . 3. This results in the formation of glutamate semialdehyde, which spontaneously cyclizes to pyrroline -5-carboxylate. 4. This enzyme begins the process of degrading proline by starting the reaction that converts it to pyrroline -5-carboxylate. 5. A deficiency of either proline oxidase or pyrroline -5-carboxylate dehydrogenase results in a buildup of proline in the body. 6. The encoded mitochondrial protein catalyzes the conversion of pyrroline -5-carboxylate to proline, which is the last step in proline biosynthesis. 7. This enzyme helps to break down the pyrroline -5-carboxylate produced in the previous reaction, converting it to the amino acid glutamate. 8. Pyrroline -5-carboxylate is further reduced by the enzyme pyrroline-5-carboxylate reductase ( P5CR ) to yield a proline amino acid.9. Pyrroline-5-carboxylate is further reduced by the enzyme pyrroline -5-carboxylate reductase ( P5CR ) to yield a proline amino acid. 10. Hyperprolinemia type II is caused by a mutation in the " ALDH4A1 " gene, for the enzyme 1-pyrroline -5-carboxylate dehydrogenase.