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...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2021-09-01
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| Series: | Frontiers in Nutrition |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fnut.2021.690359/full |
| _version_ | 1819239912923201536 |
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| author | Chelsea Truitt Wouter D. Hoff Ratnakar Deole |
| author_facet | Chelsea Truitt Wouter D. Hoff Ratnakar Deole |
| author_sort | Chelsea Truitt |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-23T13:59:40Z |
| format | Article |
| id | doaj.art-4f09527eaf2946f5afc86638d1edafa4 |
| institution | Directory Open Access Journal |
| issn | 2296-861X |
| language | English |
| last_indexed | 2024-12-23T13:59:40Z |
| publishDate | 2021-09-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Nutrition |
| spelling | doaj.art-4f09527eaf2946f5afc86638d1edafa42022-12-21T17:44:21ZengFrontiers Media S.A.Frontiers in Nutrition2296-861X2021-09-01810.3389/fnut.2021.690359690359Health Functionalities of Betaine in Patients With HomocystinuriaChelsea Truitt0Wouter D. Hoff1Ratnakar Deole2Department of Biochemistry and Microbiology, Oklahoma State University-Center for Health Sciences, Tulsa, OK, United StatesDepartment of Microbiology and Molecular Genetics, Oklahoma State University, Stillwater, OK, United StatesDepartment of Biochemistry and Microbiology, Oklahoma State University-Center for Health Sciences, Tulsa, OK, United StatesHomocystinuria 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.https://www.frontiersin.org/articles/10.3389/fnut.2021.690359/fullhomocystinuriacystathionine-β-synthasehomocysteinebetainemetabolism |
| spellingShingle | Chelsea Truitt Wouter D. Hoff Ratnakar Deole Health Functionalities of Betaine in Patients With Homocystinuria Frontiers in Nutrition homocystinuria cystathionine-β-synthase homocysteine betaine metabolism |
| 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 | homocystinuria cystathionine-β-synthase homocysteine betaine metabolism |
| url | https://www.frontiersin.org/articles/10.3389/fnut.2021.690359/full |
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