PMID: 8950194Nov 15, 1996Paper

Thiamine (vitamin B1) supplementation does not reduce fasting blood homocysteine concentration in most homozygotes for homocystinuria

Biochimica Et Biophysica Acta
D G FrankenF J Trijbels

Abstract

Homozygotes for homocystinuria due to cystathionine synthase (CS) deficiency accumulate homocysteine and methionine in their blood and tissues. High-dose pyridoxin, folic acid, vitamin B12, or betaine are therapeutical options to lower the elevated homocysteine concentration. These compounds stimulate the transsulfuration or remethylation of homocysteine. Despite such treatment, elevated blood homocysteine concentrations may persist in many homocystinurics. Therefore, it is warranted to study alternative regimen to reduce the blood homocysteine concentration in homocystinurics. Apart from entering the transsulfuration pathway, methionine can be catabolized via the transamination pathway, by conversion into 4-methylthio-2-oxobutyrate (MTOB), followed by oxidative decarboxylation of MTOB to 3-methylthiopropionate. Thiamine pyrophosphate, the active form of thiamine, is a cofactor of the supposed rate-limiting oxidative decarboxylation in the transamination of methionine. The effect of thiamine administered in 2 or 3 daily doses of 25 mg orally, was studied in nine homozygote CS deficient patients. Methionine levels decreased in 6 out of 9 patients. In 8 out of 9 patients, however, the levels of plasma homocysteine remained virtua...Continue Reading

References

Apr 1, 1983·Metabolism: Clinical and Experimental·G H BoersP W Kloppenborg
May 9, 1983·Clinica Chimica Acta; International Journal of Clinical Chemistry·A TangermanJ H van Tongeren
Mar 1, 1995·Annals of Clinical Biochemistry·M T te Poele-PothoffH J Blom

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Citations

May 31, 2012·Alcohol and Alcoholism : International Journal of the Medical Council on Alcoholism·Peter HeeseThomas Hillemacher
Jan 1, 2014·Biotechnology Progress·Timo SchmidbergerRobert Huber
Apr 19, 2002·Journal of the American Society of Nephrology : JASN·Sonja SkoupyGere Sunder-Plassmann

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