Hyperprolinemia is a disorder which can be passed on from generation to generation and is caused by an irregularity of proline metabolism. Some problems included are: seizures and psychotic disorders. There are two causes for this disorder, hyperprolinemia I, which is lacking proline oxidase which is part of the power house molecule, the mitochondria. Hyperprolinemia II, is the absence of pyrroline-5-carboxylic acid dehydrogenase activity, both defects can cause an accumulation of proline in the blood stream which eventually can lead to the rest of the body, particularly, the brain. Some patients who are affected can show symptoms while others can be asymptomatic. With increased proline levels, the NMDA and AMPA receptors are typically activated …show more content…
Higher levels of proline also are linked to impaired energy metabolic activity, which inhibits the breakdown of ATP. A well-established co-transmitter, ATP, can act as a neurotransmitter or modulator due to two different receptors, ionotropic and metabotropic. Levels of ATP are maintained using enzymes called, ectonucleotidases. These ecto-enzymes are associated with many physiological and pathological disorders. Some ecto-enzymes hydrolyze bi, tri, and monophosphonucleosides, as well as producing the final product of ATP breakdown, adenosine. Adenosine is very important in inhibited and facilitated neural connections, which is also thought of as a protective agent. The zebra fish offers multiple advantages in being a model for humans like: low maintenance, translucent embryos, rapid development and increased fecundity compared to the mammalian species. The zebra fish shares almost 80 percent homology with the human DNA which also makes this species to be a promising subject. In the brain of the zebra fish, outlines have been made with the ecto-enzymes gene