Published in Metabolism on August 01, 1980
Choline: critical role during fetal development and dietary requirements in adults. Annu Rev Nutr (2006) 2.83
Sex and menopausal status influence human dietary requirements for the nutrient choline. Am J Clin Nutr (2007) 1.76
Importance of methyl donors during reproduction. Am J Clin Nutr (2008) 1.62
Glycine N-methyltransferase and regulation of S-adenosylmethionine levels. J Biol Chem (2009) 1.55
Glycine N-methyltransferase is a folate binding protein of rat liver cytosol. Proc Natl Acad Sci U S A (1984) 1.49
Glycine N -methyltransferase deficiency: a new patient with a novel mutation. J Inherit Metab Dis (2003) 1.44
Glycine N-methyltransferase deficiency: a novel inborn error causing persistent isolated hypermethioninaemia. J Inherit Metab Dis (2001) 1.42
Dealing with methionine/homocysteine sulfur: cysteine metabolism to taurine and inorganic sulfur. J Inherit Metab Dis (2010) 1.37
Functional proteomics of nonalcoholic steatohepatitis: mitochondrial proteins as targets of S-adenosylmethionine. Proc Natl Acad Sci U S A (2003) 1.35
S-adenosylhomocysteine hydrolase deficiency in a human: a genetic disorder of methionine metabolism. Proc Natl Acad Sci U S A (2004) 1.32
Epigenetic mechanisms for nutrition determinants of later health outcomes. Am J Clin Nutr (2009) 1.28
Tissue distribution of glycine N-methyltransferase, a major folate-binding protein of liver. Proc Natl Acad Sci U S A (1994) 1.27
Perinatal choline influences brain structure and function. Nutr Rev (2006) 1.26
Homocysteine imbalance: a pathological metabolic marker. Adv Nutr (2012) 1.14
Isolated persistent hypermethioninemia. Am J Hum Genet (1995) 1.11
Transsulfuration in an adult with hepatic methionine adenosyltransferase deficiency. J Clin Invest (1988) 1.11
Sulfur as a signaling nutrient through hydrogen sulfide. Annu Rev Nutr (2014) 1.04
Gene response elements, genetic polymorphisms and epigenetics influence the human dietary requirement for choline. IUBMB Life (2007) 1.03
Inherited disorders in the conversion of methionine to homocysteine. J Inherit Metab Dis (2009) 0.97
Hepatic methionine adenosyltransferase deficiency in a 31-year-old man. Am J Hum Genet (1987) 0.93
Creatine metabolism and the consequences of creatine depletion in muscle. Mol Cell Biochem (1995) 0.92
Mechanisms and consequences of the impaired trans-sulphuration pathway in liver disease: Part I. Biochemical implications. Drugs (1990) 0.89
Mutations in the sarcosine dehydrogenase gene in patients with sarcosinemia. Hum Genet (2012) 0.84
Quantitative lipid metabolomic changes in alcoholic micropigs with fatty liver disease. Alcohol Clin Exp Res (2009) 0.83
Methoxistasis: integrating the roles of homocysteine and folic acid in cardiovascular pathobiology. Nutrients (2013) 0.83
An allosteric mechanism for switching between parallel tracks in mammalian sulfur metabolism. PLoS Comput Biol (2008) 0.82
Associations among objectively measured physical activity, fasting plasma homocysteine concentration, and MTHFR C677T genotype. Eur J Appl Physiol (2011) 0.82
Human liver methionine cycle: MAT1A and GNMT gene resequencing, functional genomics, and hepatic genotype-phenotype correlation. Drug Metab Dispos (2012) 0.81
Redox-based epigenetic status in drug addiction: a potential contributor to gene priming and a mechanistic rationale for metabolic intervention. Front Neurosci (2015) 0.80
Metabolomic profiles of arsenic (+3 oxidation state) methyltransferase knockout mice: effect of sex and arsenic exposure. Arch Toxicol (2016) 0.80
The Molecular and Cellular Effect of Homocysteine Metabolism Imbalance on Human Health. Int J Mol Sci (2016) 0.80
Genome-wide association study of selenium concentrations. Hum Mol Genet (2014) 0.79
sar: a genetic mouse model for human sarcosinemia generated by ethylnitrosourea mutagenesis. Proc Natl Acad Sci U S A (1992) 0.78
The Pediatric Methionine Requirement Should Incorporate Remethylation Potential and Transmethylation Demands. Adv Nutr (2016) 0.75
Suppression effects of betaine-enriched spinach on hyperhomocysteinemia induced by guanidinoacetic acid and choline deficiency in rats. ScientificWorldJournal (2014) 0.75
Biochemical and behavioral phenotype of AGAT and GAMT deficient mice following long-term Creatine monohydrate supplementation. Metab Brain Dis (2017) 0.75
Ultrasensitive stain for proteins in polyacrylamide gels shows regional variation in cerebrospinal fluid proteins. Science (1981) 23.67
The natural history of homocystinuria due to cystathionine beta-synthase deficiency. Am J Hum Genet (1985) 4.60
A rapid procedure for extracting genomic DNA from leukocytes. Nucleic Acids Res (1991) 3.89
Mechanism of inhibition of spinach beta-cystathionase by rhizobitoxine. Biochim Biophys Acta (1971) 3.56
Advocacy and compliance in genetic screening. Behavior of physicians and clients in a voluntary program of testing for the Tay-Sachs gene. N Engl J Med (1974) 3.47
A primate model of parkinsonism: selective destruction of dopaminergic neurons in the pars compacta of the substantia nigra by N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Proc Natl Acad Sci U S A (1983) 3.39
Transsulfuration in mammals. Microassays and tissue distributions of three enzymes of the pathway. J Biol Chem (1965) 3.21
Lemna paucicostata Hegelm. 6746: DEVELOPMENT OF STANDARDIZED GROWTH CONDITIONS SUITABLE FOR BIOCHEMICAL EXPERIMENTATION. Plant Physiol (1980) 2.76
Chronic Parkinsonism secondary to intravenous injection of meperidine analogues. Psychiatry Res (1979) 2.61
Hypophosphatemia: mouse model for human familial hypophosphatemic (vitamin D-resistant) rickets. Proc Natl Acad Sci U S A (1976) 2.59
Labile methyl balances for normal humans on various dietary regimens. Metabolism (1975) 2.53
The use of biochemical data in screening for mutant alleles and in genetic counselling. Ann Hum Genet (1974) 2.43
Homocysteine biosynthesis in green plants. Physiological importance of the transsulfuration pathway in Chlorella sorokiniana growing under steady state conditions with limiting sulfate. J Biol Chem (1978) 2.42
Sulfur-containing Compounds in Lemna perpusilla 6746 Grown at a Range of Sulfate Concentrations. Plant Physiol (1978) 2.35
Knowledge about and attitudes toward genetic screening among high-school students: the Tay-Sachs experience. Pediatrics (1977) 2.29
Beta-thalassemia disease prevention: genetic medicine applied. Am J Hum Genet (1984) 2.27
The frequency of genetic disease and congenital malformation among patients in a pediatric hospital. Can Med Assoc J (1973) 2.22
Methionine biosynthesis in lemna: inhibitor studies. Plant Physiol (1982) 2.22
The effect of Mendelian disease on human health: a measurement. Am J Med Genet (1985) 2.18
Transient tyrosinemia of the newborn: dietary and clinical aspects. Pediatrics (1967) 2.15
beta-Cystathionase In Vivo Inactivation by Rhizobitoxine and Role of the Enzyme in Methionine Biosynthesis in Corn Seedlings. Plant Physiol (1973) 2.14
Screening, counselling and treatment of hereditary metabolic disease; a survey of resources in Canada. Can Med Assoc J (1974) 2.11
Methionine Biosynthesis in Lemna: STUDIES ON THE REGULATION OF CYSTATHIONINE gamma-SYNTHASE, O-PHOSPHOHOMOSERINE SULFHYDRYLASE, AND O-ACETYLSERINE SULFHYDRYLASE. Plant Physiol (1982) 2.06
A private view of heterozygosity: eight-year follow-up study on carriers of the Tay-Sachs gene detected by high school screening in Montreal. Am J Med Genet (1984) 2.04
Alkaloids and Plant Metabolism. VII. The Kinetin-Produced Elevation in Tyramine Methylpherase Levels. Plant Physiol (1964) 2.03
Cost-benefit analysis of a thalassemia disease prevention program. Am J Public Health (1985) 1.99
The HUGO Mutation Database Initiative. Science (1998) 1.95
Twenty-year outcome analysis of genetic screening programs for Tay-Sachs and beta-thalassemia disease carriers in high schools. Am J Hum Genet (1996) 1.94
Transsulfuration in higher plants. Partial purification and properties of beta-cystathionase of spinach. Biochim Biophys Acta (1971) 1.92
Carrier screening for Tay-Sachs disease. Lancet (1990) 1.92
Quantitative analysis of pathways of methionine metabolism and their regulation in lemna. Plant Physiol (1985) 1.91
Cystathionine synthase in tissue culture derived from human skin: enzyme defect in homocystinuria. Science (1968) 1.89
Homocysteine biosynthesis in green plants. O-Phosphorylhomoserine as the physiological substrate for cystathionine gamma-synthase. J Biol Chem (1974) 1.85
On the application of knowledge to the patient with genetic disease. Prog Med Genet (1973) 1.84
Folate-responsive homocystinuria and "schizophrenia". A defect in methylation due to deficient 5,10-methylenetetrahydrofolate reductase activity. N Engl J Med (1975) 1.83
A derangement in B 12 metabolism leading to homocystinemia, cystathioninemia and methylmalonic aciduria. Biochem Biophys Res Commun (1969) 1.82
Homocystinuria associated with decreased methylenetetrahydrofolate reductase activity. Biochem Biophys Res Commun (1972) 1.79
Science's neglected legacy. Nature (2000) 1.77
Enzymes of phosphatidylcholine synthesis in lemna, soybean, and carrot. Plant Physiol (1988) 1.73
Responses of Sulfur-Containing Compounds in Lemna paucicostata Hegelm. 6746 to Changes in Availability of Sulfur Sources. Plant Physiol (1984) 1.69
Proof of "disease causing" mutation. Hum Mutat (1998) 1.68
What young people think and do when the option for cystic fibrosis carrier testing is available. J Med Genet (1993) 1.68
Consensus nomenclature for the mammalian methionine adenosyltransferase genes and gene products. Trends Genet (1997) 1.67
Genetics and Medicine: an evolving relationship. Science (1978) 1.65
Homocysteine biosynthesis in green plants: studies of the homocysteine-forming sulfhydrylase. J Biol Chem (1977) 1.64
Uptake of Amino Acids and Other Organic Compounds by Lemna paucicostata Hegelm. 6746. Plant Physiol (1985) 1.62
Gilles de la Tourette syndrome: clinical and family study of 50 cases. Ann Neurol (1980) 1.59
Pharmacologic distinction of different orthostatic hypotension syndromes. Neurology (1981) 1.59
Aggression, suicide, and serotonin: relationships to CSF amine metabolites. Am J Psychiatry (1982) 1.58
A study of cardiovascular risk in heterozygotes for homocystinuria. Am J Hum Genet (1981) 1.54
Sulfate Uptake and Its Regulation in Lemna paucicostata Hegelm. 6746. Plant Physiol (1984) 1.53
Trans-sulfuration in mammals. The methionine-sparing effect of cystine. J Biol Chem (1967) 1.52
Phosphatidylcholine synthesis: differing patterns in soybean and carrot. Plant Physiol (1988) 1.50
Recommendations for locus-specific databases and their curation. Hum Mutat (2008) 1.49
Loss of a parathyroid hormone-sensitive component of phosphate transport in X-linked hypophosphatemia. Science (1972) 1.48
Recycling of methionine sulfur in a higher plant by two pathways characterized by either loss or retention of the 4-carbon moiety. Biochem Biophys Res Commun (1981) 1.47
Use of phosphate and vitamin D to prevent dwarfism and rickets in X-linked hypophosphatemia. N Engl J Med (1972) 1.46
Renal handling of phosphate in vivo and in vitro by the X-linked hypophosphatemic male mouse: evidence for a defect in the brush border membrane. Kidney Int (1978) 1.45
In vivo regulation of de novo methionine biosynthesis in a higher plant (lemna). Plant Physiol (1985) 1.45
The defect in transcellular transport of phosphate in the nephron is located in brush-border membranes in X-linked hypophosphatemia (Hyp mouse model). Can J Biochem (1978) 1.45
Regulatory Structure of the Biosynthetic Pathway for the Aspartate Family of Amino Acids in Lemna paucicostata Hegelm. 6746, with Special Reference to the Role of Aspartokinase. Plant Physiol (1989) 1.44
Glycine N -methyltransferase deficiency: a new patient with a novel mutation. J Inherit Metab Dis (2003) 1.44
Homocystinuria due to cystathionine synthase deficiency: the effect of pyridoxine. J Clin Invest (1970) 1.43
Neonatal hypertyrosinemia and evidence for deficiency of ascorbic acid in Arctic and subarctic peoples. Can Med Assoc J (1975) 1.42
Novel PKU mutation on haplotype 2 in French-Canadians. Am J Hum Genet (1989) 1.40
Serum 1,25-dihydroxyvitamin D levels in normal subjects and in patients with hereditary rickets or bone disease. N Engl J Med (1978) 1.40
Homocystinuria: studies in tissue culture. Pediatr Res (1973) 1.39
[Tay-Sachs disease: prenatal detection and diagnosis]. Union Med Can (1972) 1.36
Lemna paucicostata Hegelm. 6746: LIFE CYCLE AND CHARACTERIZATION OF THE COLONY TYPES IN A POPULATION. Plant Physiol (1980) 1.36
Homoserine esterification in green plants. Plant Physiol (1974) 1.35
Endogenous renal clearance rates of free amino acids in pre-pubertal children. (Employing an accelerated procedure for elution chromatography of basic amino acids on ion exchange resin). Pediatrics (1965) 1.34
Altered gastric emptying and secretion in primary anorexia nervosa. Gastroenterology (1979) 1.33
Phenylketonuria: epitome of human biochemical genetics (first of two parts). N Engl J Med (1980) 1.30
Genetic variants of Tay-Sachs disease: Tay-Sachs disease and Sandhoff's disease in French Canadians, juvenile Tay-Sachs disease in Lebanese Canadians, and a Tay-Sachs screening program in the French-Canadian population. Prog Clin Biol Res (1977) 1.30
Use of dithiothreitol to correct cystine storage in cultured cystinotic fibroblasts. Lancet (1970) 1.30
Thiamine-responsive maple-syrup-urine disease. Lancet (1971) 1.29
Familial cold urticaria. Clin Exp Dermatol (1993) 1.29
Hyperparathyroidism as the cause of hyperaminoaciduria and phosphaturia in human vitamin D deficiency. Pediatr Res (1967) 1.29
Recurrent mutation, gene conversion, or recombination at the human phenylalanine hydroxylase locus: evidence in French-Canadians and a catalog of mutations. Am J Hum Genet (1990) 1.28
A derangement in B12 metabolism associated with homocystinemia, cystathioninemia, hypomethioninemia and methylmalonic aciduria. Am J Med (1970) 1.28
Defective metabolism of vitamin B 12 in fibroblasts from children with methylmalonicaciduria. Biochem Biophys Res Commun (1971) 1.28
Pyridoxine-responsive genetic disease. Fed Proc (1971) 1.28
Phenylketonuria and other phenylalanine hydroxylation mutants in man. Annu Rev Genet (1980) 1.27
Management of hereditary metabolic disease. The role of allied health personnel. N Engl J Med (1971) 1.26
A different approach to treatment of phenylketonuria: phenylalanine degradation with recombinant phenylalanine ammonia lyase. Proc Natl Acad Sci U S A (1999) 1.25
Human phenylalanine hydroxylase mutations and hyperphenylalaninemia phenotypes: a metanalysis of genotype-phenotype correlations. Am J Hum Genet (1997) 1.25
In vivo metabolism of 5'-methylthioadenosine in lemna. Plant Physiol (1983) 1.24
Homocysteine Biosynthesis in Green Plants: Physiological Importance of the Transsulfuration Pathway in Lemna paucicostata. Plant Physiol (1981) 1.24
Sulfite oxidase deficiency in man: demonstration of the enzymatic defect. Science (1967) 1.23
Synthesis of homocysteine and cysteine by enzyme extracts of spinach. Biochem Biophys Res Commun (1967) 1.23
Outcome of early and long-term management of classical maple syrup urine disease. Pediatrics (1981) 1.22
PAH Mutation Analysis Consortium Database: a database for disease-producing and other allelic variation at the human PAH locus. Nucleic Acids Res (1996) 1.21
The characterization of hereditary abnormalities of keratin: Clouston's ectodermal dysplasia. Birth Defects Orig Artic Ser (1971) 1.20
Threonine Synthase of Lemna paucicostata Hegelm. 6746. Plant Physiol (1984) 1.20
Phytostat for the growth of lemna in semicontinuous culture with low sulfate. Plant Physiol (1978) 1.20
Diagnosis and treatment: interpreting the positive screening test in the newborn infant. Pediatrics (1967) 1.20
Prevention of mental retardation in offspring of hyperphenylalaninemic mothers. Am J Public Health (1982) 1.19
Heterogeneity in genetic control of phenylalanine metabolism in man. Nature (1968) 1.19
Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease. Metabolism (1974) 1.18
Methionine Synthesis in Lemna: Inhibition of Cystathionine gamma-Synthase by Propargylglycine. Plant Physiol (1982) 1.18