PubRank

S H Mudd

Author PubWeight™ 123.88‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 The natural history of homocystinuria due to cystathionine beta-synthase deficiency. Am J Hum Genet 1985 4.60
2 Mechanism of inhibition of spinach beta-cystathionase by rhizobitoxine. Biochim Biophys Acta 1971 3.56
3 Transsulfuration in mammals. Microassays and tissue distributions of three enzymes of the pathway. J Biol Chem 1965 3.21
4 Lemna paucicostata Hegelm. 6746: DEVELOPMENT OF STANDARDIZED GROWTH CONDITIONS SUITABLE FOR BIOCHEMICAL EXPERIMENTATION. Plant Physiol 1980 2.76
5 Labile methyl balances for normal humans on various dietary regimens. Metabolism 1975 2.53
6 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
7 Sulfur-containing Compounds in Lemna perpusilla 6746 Grown at a Range of Sulfate Concentrations. Plant Physiol 1978 2.35
8 Methionine biosynthesis in lemna: inhibitor studies. Plant Physiol 1982 2.22
9 beta-Cystathionase In Vivo Inactivation by Rhizobitoxine and Role of the Enzyme in Methionine Biosynthesis in Corn Seedlings. Plant Physiol 1973 2.14
10 Methionine Biosynthesis in Lemna: STUDIES ON THE REGULATION OF CYSTATHIONINE gamma-SYNTHASE, O-PHOSPHOHOMOSERINE SULFHYDRYLASE, AND O-ACETYLSERINE SULFHYDRYLASE. Plant Physiol 1982 2.06
11 Alkaloids and Plant Metabolism. VII. The Kinetin-Produced Elevation in Tyramine Methylpherase Levels. Plant Physiol 1964 2.03
12 Transsulfuration in higher plants. Partial purification and properties of beta-cystathionase of spinach. Biochim Biophys Acta 1971 1.92
13 Quantitative analysis of pathways of methionine metabolism and their regulation in lemna. Plant Physiol 1985 1.91
14 Cystathionine synthase in tissue culture derived from human skin: enzyme defect in homocystinuria. Science 1968 1.89
15 Homocysteine biosynthesis in green plants. O-Phosphorylhomoserine as the physiological substrate for cystathionine gamma-synthase. J Biol Chem 1974 1.85
16 Folate-responsive homocystinuria and "schizophrenia". A defect in methylation due to deficient 5,10-methylenetetrahydrofolate reductase activity. N Engl J Med 1975 1.83
17 A derangement in B 12 metabolism leading to homocystinemia, cystathioninemia and methylmalonic aciduria. Biochem Biophys Res Commun 1969 1.82
18 Homocystinuria associated with decreased methylenetetrahydrofolate reductase activity. Biochem Biophys Res Commun 1972 1.79
19 Enzymes of phosphatidylcholine synthesis in lemna, soybean, and carrot. Plant Physiol 1988 1.73
20 Responses of Sulfur-Containing Compounds in Lemna paucicostata Hegelm. 6746 to Changes in Availability of Sulfur Sources. Plant Physiol 1984 1.69
21 Consensus nomenclature for the mammalian methionine adenosyltransferase genes and gene products. Trends Genet 1997 1.67
22 Homocysteine biosynthesis in green plants: studies of the homocysteine-forming sulfhydrylase. J Biol Chem 1977 1.64
23 Uptake of Amino Acids and Other Organic Compounds by Lemna paucicostata Hegelm. 6746. Plant Physiol 1985 1.62
24 A study of cardiovascular risk in heterozygotes for homocystinuria. Am J Hum Genet 1981 1.54
25 Sulfate Uptake and Its Regulation in Lemna paucicostata Hegelm. 6746. Plant Physiol 1984 1.53
26 Trans-sulfuration in mammals. The methionine-sparing effect of cystine. J Biol Chem 1967 1.52
27 Phosphatidylcholine synthesis: differing patterns in soybean and carrot. Plant Physiol 1988 1.50
28 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
29 In vivo regulation of de novo methionine biosynthesis in a higher plant (lemna). Plant Physiol 1985 1.45
30 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
31 Glycine N -methyltransferase deficiency: a new patient with a novel mutation. J Inherit Metab Dis 2003 1.44
32 Homocystinuria due to cystathionine synthase deficiency: the effect of pyridoxine. J Clin Invest 1970 1.43
33 Homocystinuria: studies in tissue culture. Pediatr Res 1973 1.39
34 Lemna paucicostata Hegelm. 6746: LIFE CYCLE AND CHARACTERIZATION OF THE COLONY TYPES IN A POPULATION. Plant Physiol 1980 1.36
35 Homoserine esterification in green plants. Plant Physiol 1974 1.35
36 Labile methyl group balances in the human: the role of sarcosine. Metabolism 1980 1.34
37 A derangement in B12 metabolism associated with homocystinemia, cystathioninemia, hypomethioninemia and methylmalonic aciduria. Am J Med 1970 1.28
38 Defective metabolism of vitamin B 12 in fibroblasts from children with methylmalonicaciduria. Biochem Biophys Res Commun 1971 1.28
39 Pyridoxine-responsive genetic disease. Fed Proc 1971 1.28
40 In vivo metabolism of 5'-methylthioadenosine in lemna. Plant Physiol 1983 1.24
41 Homocysteine Biosynthesis in Green Plants: Physiological Importance of the Transsulfuration Pathway in Lemna paucicostata. Plant Physiol 1981 1.24
42 Sulfite oxidase deficiency in man: demonstration of the enzymatic defect. Science 1967 1.23
43 Synthesis of homocysteine and cysteine by enzyme extracts of spinach. Biochem Biophys Res Commun 1967 1.23
44 Threonine Synthase of Lemna paucicostata Hegelm. 6746. Plant Physiol 1984 1.20
45 Phytostat for the growth of lemna in semicontinuous culture with low sulfate. Plant Physiol 1978 1.20
46 Methionine Synthesis in Lemna: Inhibition of Cystathionine gamma-Synthase by Propargylglycine. Plant Physiol 1982 1.18
47 Demyelination of the brain is associated with methionine adenosyltransferase I/III deficiency. J Clin Invest 1996 1.18
48 Uptake of Choline and Ethanolamine by Lemna paucicostata Hegelm. 6746. Plant Physiol 1986 1.17
49 Deranged B 12 metabolism: studies of fibroblasts grown in tissue culture. Biochem Med 1970 1.17
50 Homocystinuria due to cystathionine synthase deficiency. Studies of nitrogen balance and sulfur excretion. J Clin Invest 1975 1.15
51 Enzymatic synthesis of cystathionine by extracts of spinach, requiring O-acetylhomoserine or O-succinylhomoserine. Biochem Biophys Res Commun 1966 1.15
52 Methionine adenosyltransferase I/III deficiency: novel mutations and clinical variations. Am J Hum Genet 2000 1.15
53 Homocystinuria due to cystathionine synthase deficiency: the metabolism of L-methionine. J Clin Invest 1965 1.14
54 Sulfuration of O-acetylhomoserine and O-acetylserine by two enzyme fractions from spinach. Biochem Biophys Res Commun 1968 1.14
55 In Vivo Regulation of Threonine and Isoleucine Biosynthesis in Lemna paucicostata Hegelm. 6746. Plant Physiol 1988 1.12
56 Homocystinuria with methylmalonic aciduria: two cases in a sibship. Biochem Med 1970 1.11
57 Transsulfuration in an adult with hepatic methionine adenosyltransferase deficiency. J Clin Invest 1988 1.11
58 Dominant inheritance of isolated hypermethioninemia is associated with a mutation in the human methionine adenosyltransferase 1A gene. Am J Hum Genet 1997 1.10
59 Molecular mechanisms of an inborn error of methionine pathway. Methionine adenosyltransferase deficiency. J Clin Invest 1995 1.05
60 Effects of Orthophosphate and Adenosine 5'-Phosphate on Threonine Synthase and Cystathionine gamma-Synthate of Lemna paucicostata Hegelm. 6746. Plant Physiol 1986 1.04
61 S-Adenosylhomocysteine hydrolase deficiency: a second patient, the younger brother of the index patient, and outcomes during therapy. J Inherit Metab Dis 2005 1.03
62 S-adenosylhomocysteine hydrolase deficiency in a 26-year-old man. J Inherit Metab Dis 2006 1.03
63 Biochemical basis for the dominant inheritance of hypermethioninemia associated with the R264H mutation of the MAT1A gene. A monomeric methionine adenosyltransferase with tripolyphosphatase activity. J Biol Chem 2001 1.00
64 Deranged B 12 metabolism: effects on sulfur amino acid metabolism. Biochem Med 1970 1.00
65 Vascular disease and homocysteine metabolism. N Engl J Med 1985 0.97
66 Hypermethioninaemia due to methionine adenosyltransferase I/III (MAT I/III) deficiency: diagnosis in an expanded neonatal screening programme. J Inherit Metab Dis 2008 0.96
67 Mental retardation, megaloblastic anaemia, methylmalonic aciduria and abnormal homocysteine metabolism due to an error in vitamin B12 metabolism. Clin Sci Mol Med 1974 0.96
68 Deficiencies of cystathionase and homoserine dehydratase activities in cystathioninuria. Proc Natl Acad Sci U S A 1966 0.95
69 Adaptation of Lemna paucicostata to Sublethal Methionine Deprivation. Plant Physiol 1983 0.94
70 Cobalamins in fibroblasts cultured from normal control subjects and patients with methylmalonic aciduria. Pediatr Res 1976 0.93
71 Hepatic methionine adenosyltransferase deficiency in a 31-year-old man. Am J Hum Genet 1987 0.93
72 Aspartokinase of Lemna paucicostata Hegelm. 6746. Plant Physiol 1989 0.90
73 Persistent hypermethioninaemia with dominant inheritance. J Inherit Metab Dis 1992 0.87
74 Plasma homocyst(e)ine or homocysteine? N Engl J Med 1995 0.86
75 Radioactive methionine: determination, and distribution of radioactivity in the sulfur, methyl and 4-carbon moieties. J Biochem Biophys Methods 1985 0.85
76 Methionine transamination in patients with homocystinuria due to cystathionine beta-synthase deficiency. Metabolism 2000 0.85
77 Maternal gamma-cystathionase deficiency: absence of both teratogenic effects and pregnancy complications. Am J Obstet Gynecol 1999 0.83
78 Platelet survival and morphology in homocystinuria due to cystathionine synthase deficiency. N Engl J Med 1976 0.83
79 Phosphatidylcholine synthesis in the rat: the substrate for methylation and regulation by choline. Lipids 1990 0.82
80 The conformation of ribonucleosides in solution. The effect of structure on the orientation of the base. Biochemistry 1967 0.82
81 Threonine dehydratase activity in humans lacking cystathionine synthase. Biochem Biophys Res Commun 1965 0.80
82 Cardiovascular risk in heterozygotes for homocystinuria. Am J Hum Genet 1982 0.80
83 Methionine adenosyltransferase I/III deficiency: two Korean compound heterozygous siblings with a novel mutation. J Inherit Metab Dis 2002 0.79
84 Affective disorders and S-adenosylmethionine: a new hypothesis. Trends Neurosci 1989 0.79
85 N-5,10-methylenetetrahydrofolate reductase deficiency and schizophrenia: a working hypothesis. J Psychiatr Res 1974 0.78
86 Enthalpy changes accompanying the transfer of a methyl group from S-adenosylmethionine and other sulfonium compounds to homocysteine. Biochemistry 1966 0.77
87 Cystathioninuria and homocystinuria. Clin Chim Acta 1975 0.77
88 Preparation of cystathionine sulfoxide and sulfone and some properties relating to their differentiation. Anal Biochem 1975 0.76
89 Epileptiform ocular movements with methylmalonic aciduria and homocystinuria. Am J Ophthalmol 1980 0.76
90 Pregnancy and thrombophlebitis in homocystinuria. Blood 1980 0.75
91 Inborn errors of metabolism. Vitamin-responsive genetic disease. J Clin Pathol Suppl (R Coll Pathol) 1974 0.75
92 Pulmonary embolism in a homocystinuric patient during treatment with dipyridamole and acetylsalicylic acid. N Engl J Med 1978 0.75
93 Cystathioninuria. Am J Dis Child 1968 0.75
94 Vitamin-responsive genetic abnormalities. Adv Nutr Res 1982 0.75
95 A sensitive and specific assay for cystathionine: cystathionine content of several plant tissues. Anal Biochem 1974 0.75
96 Diseases of sulphur metabolism: implications for the methionine-homocysteine cycle, and vitamin responsiveness. Ciba Found Symp 1979 0.75
97 Determination of sulfite in biological fluids. The usefulness of radioactive N-ethylmaleimide. Anal Biochem 1968 0.75