Effect of betaine on<i>S</i>‐adenosylmethionine levels in the cerebrospinal fluid in a patient with methylenetetrahydrofolate reductase deficiency and peripheral neuropathy

Kishi, Takamasa; Kawamura, Isao; Harada, Yosuke; Eguchi, Takaatsu; Sakura, Nobuo; Ueda, Kazuhiro; Narisawa, Kuniaki; Rosenblatt, David S.

doi:10.1007/bf00711591

Cited by 30 publications

(25 citation statements)

References 15 publications

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“…Also all three patients had other electrophysiological evidence of generalised peripheral nerve involvement. As was seen in patients 1 and 2 here, worsening lower limb neuropathy can herald potential respiratory decompensation (Nishimura et al 1985;Kishi et al 1994). The response of this peripheral neuropathy is often at best limited (Chaabene-Masmoudi et al 2009;Schiff et al 2011); however, in this case series complete electrophysiological resolution was documented in the infantile onset MTHFR case accompanied by normalisation of clinical symptomology, while apparent clinical resolution was seen in patients 1 and 2.…”

Section: Discussionsupporting

confidence: 51%

Reversal of Respiratory Failure in Both Neonatal and Late Onset Isolated Remethylation Disorders

et al. 2014

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Respiratory failure is a well-documented potential presentation of inherited isolated remethylation disorders (IRDs). It appears to be a combination of both central and peripheral neuropathy and has previously often been considered to herald an irreversible neurological decline. We present three patients, one with methionine synthase (cblG) and two with methyltetrahydrofolate reductase deficiency (MTHFR). One patient with MTHFR presented in infancy, and other patients in later childhood. All three patients required intubation for respiratory failure but in all three, this was totally reversed by the initiation of medical therapy. This consisted of betaine and folinic acid supplementation in all three, methionine in two and cobalamin supplementation in two. The rate of respiratory improvement was variable, though two of the cases were successful extubated within a week of commencement of medical therapy. We document their subsequent clinical, biochemical and electrophysiological progress and review the potential pathological mechanisms underlying respiratory failure in these disorders.

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Section: Discussionsupporting

confidence: 51%

Reversal of Respiratory Failure in Both Neonatal and Late Onset Isolated Remethylation Disorders

et al. 2014

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“…Thus, it is a nutrient that plays an important role in the health of the cardiovascular system. Studies have suggested that betaine helps to reduce potentially toxic levels of homocysteine, a naturally occurring amino acid that can be harmful to blood vessels, thereby contributing to the development of heart disease, stroke, and Alzheimer's disease (2)(3)(4). In addition, betaine has been shown to enhance the expression and activity of key participants in vascular inflammation related to deteriorated vessel function, such as hyperosmotic cell shrinkage or hypoosmotic cell swelling in liver and kidney (5,6).…”

mentioning

confidence: 99%

Age-Related Differences in the Force Generation Capabilities and Tendon Extensibilities of Knee Extensors and Plantar Flexors in Men

Kubo

Ishida²,

Komuro³

et al. 2007

The Journals of Gerontology Series A: Biological Sciences and M

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Betaine is an important human nutrient obtained from various foods. In the present study, we assessed the anti-inflammatory effect of betaine on nuclear factor-jB (NF-jB) during aging. Sprague-Dawley (SD) rats, ages 7 and 21 months, were used in this study. The older rats were fed betaine. To elucidate the effect of betaine on oxidative stress-induced NF-jB and its signaling pathway at molecular levels, YPEN-1 cells were used. Results showed that betaine suppressed NF-jB and its related gene expressions of cyclooxygenase-2 (COX-2), inducible nitric oxide synthase (iNOS), vascular cell adhesion molecule-1 (VCAM-1), and intracellular cell adhesion molecule-1 (ICAM-1) in aged kidney. Furthermore, betaine attenuated oxidative stress-induced NF-jB via nuclear factor-inducing kinase/IjB kinase (NIK/IKK) and mitogen-activated protein kinases (MAPKs) in the YPEN-1 cells. On the basis of these results, we concluded that betaine suppressed the age-related NF-jB activities associated with upregulated NIK/IKK and MAPKs that were induced by oxidative stress. Thus, betaine might be useful as a preventive agent against the activation of NF-jB induced during inflammation and aging.

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“…Pooled data from many studies showed that mild homocysteinemia occurs in 9-42% of subjects below 50 years of age who have peripheral or cerebral occlusive arterial disease, myocardial infarction, or thromboembolism [23]. About 21% of young patients with coronary artery disease, 24% of patients with cerebrovascular disease, and 32% of patients with peripheral vascular disease are found to have mild postmethionine homocysteinemia.…”

Section: Mild Homocysteinemiamentioning

confidence: 99%

Betaine Supplementation for Various Clinical Disorders

Jayalakshmi

Vanitha

2017

Asian J Pharm Clin Res

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Betaine is distributed widely in animals, plants, and microorganisms and rich dietary sources include seafood, especially marine invertebrates. Betaine is N-trimethylated amino acid called as glycine betaine. It is a by-product. Betaine aldehyde is produced when choline dehydrogenase acts on choline, then betaine aldehyde is oxidized to form betaine by aldehyde dehydrogenase. Metabolic derived betaines possess various functions in our body in which they act as methyl donor which helps in liver function, detoxication, and cellular functions. It plays an important role in fat metabolism. Recent research found that betaine can convert homocysteine to cysteine thus they prevent heart disease. Choline is oxidized to betaine in liver and kidney. Intracellular betaine serves as an osmolyte that regulates cell volume and tissue integrity. Betaine not only plays as an osmolyte but also play a major role in the protection of the liver and other tissues. Consequently, it has been proposed that betaine has significant nutrient for prevention of chronic disease. Betaine has been shown to protect internal organs, improve vascular risk factors, and enhance performance. Databases of betaine content in food are being developed for correlation with population health studies. This review focuses on the aspects of wide research field with emphasis on a recent data relevant to various human diseases.

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Effect of betaine onS‐adenosylmethionine levels in the cerebrospinal fluid in a patient with methylenetetrahydrofolate reductase deficiency and peripheral neuropathy

Cited by 30 publications

References 15 publications

Reversal of Respiratory Failure in Both Neonatal and Late Onset Isolated Remethylation Disorders

Reversal of Respiratory Failure in Both Neonatal and Late Onset Isolated Remethylation Disorders

Age-Related Differences in the Force Generation Capabilities and Tendon Extensibilities of Knee Extensors and Plantar Flexors in Men

Betaine Supplementation for Various Clinical Disorders

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