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Hyperprolinemia can also occur with other conditions, such as malnutrition or liver disease. In particular, individuals with conditions that cause elevated levels of lactic acid in the blood, such as lactic acidemia, are likely to have elevated proline levels, because lactic acid inhibits the breakdown of proline. [1]
Other hydroxyprolines also exist in nature. The most notable ones are 2,3-cis-, 3,4-trans-, and 3,4-dihydroxyproline, which occurs in diatom cell walls [12] and are postulated to have a role in silica deposition. Hydroxyproline is also found in the walls of oomycetes, fungus-like protists related to diatoms.
In medicine, the presence of elevated transaminases, commonly the transaminases alanine transaminase (ALT) and aspartate transaminase (AST), may be an indicator of liver dysfunction. [ 1 ] [ 2 ] Other terms include transaminasemia , [ 3 ] and elevated liver enzymes (though they are not the only enzymes in the liver).
Hence, this enzyme has one substrate, trans-4-hydroxy-L-proline, and one product, cis-4-hydroxy-D-proline. This enzyme belongs to the family of isomerases, specifically those racemases and epimerases acting on amino acids and derivatives. The systematic name of this enzyme class is 4-hydroxyproline 2-epimerase.
Ramaswamy SG (1983). "Conversion of 3-hydroxyproline to proline in the rat requires reduced pyridine-nucleotides". Fed. Proc. 42: 2232. Visser WF, Verhoeven-Duif NM, de Koning TJ (June 2012). "Identification of a human trans-3-hydroxy-L-proline dehydratase, the first characterized member of a novel family of proline racemase-like enzymes".
Laboratory investigations revealed elevated lysine, low levels of carnitine and an abnormal acylcarnitine profile in urine and blood. The abnormal acylcarnitine species was eventually identified as 2-trans,4-cis-decadienoylcarnitine, an intermediate of linoleic acid metabolism. [1] The index case died of respiratory failure at four months of age.
Hypoxia-inducible factor-proline dioxygenase contains iron, and requires ascorbate. Hypoxia-inducible factor (HIF) is an evolutionarily conserved transcription factor [ 7 ] that allows the cell to respond physiologically to low concentrations of oxygen. [ 8 ]
This enzyme is also called hydroxy-L-proline oxidase. This enzyme was originally thought to participate in the metabolism of arginine and proline . However, recent data show that it is unlikely since neither 4-oxo-L-proline nor cis -4-hydroxy-L-proline are metabolites of these metabolic pathways.