What does prolidase deficiency mean?
Definitions for prolidase deficiency
pro·l·i·dase defi·cien·cy
This dictionary definitions page includes all the possible meanings, example usage and translations of the word prolidase deficiency.
Wikipedia
Prolidase deficiency
Prolidase deficiency (PD) is an extremely uncommon autosomal recessive disorder associated with collagen metabolism that affects connective tissues and thus a diverse array of organ systems more broadly, though it is extremely inconsistent in its expression. Collagen is a structural protein found i.a. in bone, skin and connective tissues that is broken down into iminodipeptides at the end of its lifecycle. Of these dipeptides, those containing C-terminal proline or hydroxyproline would normally be broken down further by the enzyme Prolidase, recovering and thus recycling the constituent amino acids. Due to a genetic defect, prolidase activity in individuals with PD is either knocked out or severely reduced. Those affected therefore eliminate excessive amounts of iminodipeptides in their urine, wasting this precious resource, with debilitating effects.
Wikidata
Prolidase deficiency
Prolidase deficiency is an autosomal recessive disease that is extremely uncommon and is associated with collagen metabolism and affects the connective tissues. This rare condition affects assorted systems because of an error on an enzyme, which is affiliated with the creation of collagen. Collagen is a powerful protein that is observed in the bones, tissues, and the skin. Collagen causes the degradation of iminodipeptides. Iminodipeptides are compounds, which break down to form amino acids. Prolidase is an enzyme responsible for the breaking down of iminodipeptides. In this condition there is a defect, which causes a lack of prolidase. Those affected by PD eliminate large quantities of iminodipeptides in their urine because of a deficiency in the prolidase enzyme. When the function of prolidase operates incorrectly it results in this recessive connective tissue condition. Prolidase splits dipeptides that contain C-terminal proline or hydroxyproline. When the amount of prolidase is insufficient, the typical recycling of the residues of proline, acquired from the degrading of collagen, is not functioning properly. As a result, there is an accumulation of iminodipeptides causes disruptions in the connective tissue metabolism and elimination of an excessive volume of iminodipeptides in the urine.
U.S. National Library of Medicine
Prolidase Deficiency
Rare autosomal recessive disorder of metabolism due to mutations in the prolidase gene. It is characterized by recurrent lower extremity skin ulcers, recurrent infections, and FACIES, often with mental retardation.
Numerology
Chaldean Numerology
The numerical value of prolidase deficiency in Chaldean Numerology is: 7
Pythagorean Numerology
The numerical value of prolidase deficiency in Pythagorean Numerology is: 2
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"prolidase deficiency." Definitions.net. STANDS4 LLC, 2024. Web. 28 Apr. 2024. <https://www.definitions.net/definition/prolidase+deficiency>.
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