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Health Functionalities of Betaine in Patients With Homocystinuria

Homocystinuria is a medical condition that can have widespread and harmful effects on multiple organ systems within the body. This disease is caused by a deficiency in one of the enzymes involved in the methionine metabolism pathway. One example would be a deficiency in cystathionine-β-synthase (CBS...

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Autores principales: Truitt, Chelsea, Hoff, Wouter D., Deole, Ratnakar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8459993/
https://www.ncbi.nlm.nih.gov/pubmed/34568401
http://dx.doi.org/10.3389/fnut.2021.690359
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author Truitt, Chelsea
Hoff, Wouter D.
Deole, Ratnakar
author_facet Truitt, Chelsea
Hoff, Wouter D.
Deole, Ratnakar
author_sort Truitt, Chelsea
collection PubMed
description Homocystinuria is a medical condition that can have widespread and harmful effects on multiple organ systems within the body. This disease is caused by a deficiency in one of the enzymes involved in the methionine metabolism pathway. One example would be a deficiency in cystathionine-β-synthase (CBS), which is seen in classical homocystinuria. A deficiency in CBS can lead to elevated levels of homocysteine (HCY) and possible depletion of methionine and/or cysteine. There are several different treatment options for patients with this condition, one of which is the administration of the drug betaine. Here we review the use of betaine to decrease these elevated levels of homocysteine back to within normal ranges. Published literature indicates that the use of this choline derivative is most beneficial to patients who are either not compliant with the recommended low methionine and low protein diet or wish to consume a less restricted diet.
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spelling pubmed-84599932021-09-24 Health Functionalities of Betaine in Patients With Homocystinuria Truitt, Chelsea Hoff, Wouter D. Deole, Ratnakar Front Nutr Nutrition Homocystinuria is a medical condition that can have widespread and harmful effects on multiple organ systems within the body. This disease is caused by a deficiency in one of the enzymes involved in the methionine metabolism pathway. One example would be a deficiency in cystathionine-β-synthase (CBS), which is seen in classical homocystinuria. A deficiency in CBS can lead to elevated levels of homocysteine (HCY) and possible depletion of methionine and/or cysteine. There are several different treatment options for patients with this condition, one of which is the administration of the drug betaine. Here we review the use of betaine to decrease these elevated levels of homocysteine back to within normal ranges. Published literature indicates that the use of this choline derivative is most beneficial to patients who are either not compliant with the recommended low methionine and low protein diet or wish to consume a less restricted diet. Frontiers Media S.A. 2021-09-09 /pmc/articles/PMC8459993/ /pubmed/34568401 http://dx.doi.org/10.3389/fnut.2021.690359 Text en Copyright © 2021 Truitt, Hoff and Deole. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Nutrition
Truitt, Chelsea
Hoff, Wouter D.
Deole, Ratnakar
Health Functionalities of Betaine in Patients With Homocystinuria
title Health Functionalities of Betaine in Patients With Homocystinuria
title_full Health Functionalities of Betaine in Patients With Homocystinuria
title_fullStr Health Functionalities of Betaine in Patients With Homocystinuria
title_full_unstemmed Health Functionalities of Betaine in Patients With Homocystinuria
title_short Health Functionalities of Betaine in Patients With Homocystinuria
title_sort health functionalities of betaine in patients with homocystinuria
topic Nutrition
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8459993/
https://www.ncbi.nlm.nih.gov/pubmed/34568401
http://dx.doi.org/10.3389/fnut.2021.690359
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