Betaine dose and treatment intervals in therapy for homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency

Journal of Inherited Metabolic Disease

Volumn 21, Issue 1, 1998, Pages 84-85

  Sakura, N ^a   Ono, H ^a   Nomura, S ^a   Ueda, H ^a   Fujita, N ^a  

^a HIROSHIMA UNIV SCHOOL OF MEDICINE   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

5,10 METHYLENETETRAHYDROFOLATE REDUCTASE (FADH2); BETAINE; HOMOCYSTEINE;

ADULT; ARTICLE; CASE REPORT; CLINICAL TRIAL; CONTROLLED STUDY; DRUG BLOOD LEVEL; DRUG EFFICACY; DRUG HALF LIFE; ENZYME ACTIVITY; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HOMOCYSTINURIA; HUMAN; ORAL DRUG ADMINISTRATION;

5,10-METHYLENETETRAHYDROFOLATE REDUCTASE (FADH2); ADULT; BETAINE; DOSE-RESPONSE RELATIONSHIP, DRUG; DRUG ADMINISTRATION SCHEDULE; HOMOCYSTINURIA; HUMANS; METHYLENETETRAHYDROFOLATE REDUCTASE (NADPH2); OXIDOREDUCTASES;

EID: 0031985357     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1005331902497     Document Type: Article

References (4)

1. Allen RH, Stabler SP, Lindenbaum J (1993) Serum betaine, N,N-dimethylglycine and N-methylglycine levels in patients with cobalamin and folate deficiency and related inborn errors of metabolism. Metabolism 42: 1448-1460.
2. Kishi T, Kawamura I, Harada Y, et al (1994) Effect of betaine on S-adenosylmethionine levels in the cerebrospinal fluid in a patient with methylenetetrahydrofolate reductase deficiency and peripheral neuropathy. J Inher Metab Dis 17: 560-565.
3. Sakura N, Ono H, Ueda H, Nomura S (1997) High performance liquid Chromatographic determination of human serum betaine and its clinical trial in the betaine treatment of homocystinuria (in Japanese with English abstract). J Jpn Pediatr Soc 101: 25-29.
4. Skiba WE, Taylor MP, Wells MS, Mangum JH, Awad WM (1982) Human hepatic methionine biosynthesis. J Biol Chem 257: 14944-14948.