Rank |
Title |
Journal |
Year |
PubWeight™‹?› |
1
|
Inborn errors of metabolism. Sensitivity of screening tests in high risk patients.
|
Clin Pediatr (Phila)
|
1989
|
1.38
|
2
|
Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake.
|
J Inherit Metab Dis
|
2008
|
1.13
|
3
|
Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient mice.
|
J Inherit Metab Dis
|
2007
|
1.06
|
4
|
Selective screening of 10,000 high-risk Brazilian patients for the detection of inborn errors of metabolism.
|
Eur J Pediatr
|
1997
|
0.99
|
5
|
Inhibition of the mitochondrial respiratory chain complex activities in rat cerebral cortex by methylmalonic acid.
|
Neurochem Int
|
2002
|
0.92
|
6
|
Evidence that oxidative stress is increased in patients with X-linked adrenoleukodystrophy.
|
Biochim Biophys Acta
|
2004
|
0.92
|
7
|
Reduction of large neutral amino acid levels in plasma and brain of hyperleucinemic rats.
|
Neurochem Int
|
2001
|
0.92
|
8
|
Oxidative stress in patients with phenylketonuria.
|
Biochim Biophys Acta
|
2005
|
0.85
|
9
|
Pharmacological evidence for GABAergic and glutamatergic involvement in the convulsant and behavioral effects of glutaric acid.
|
Brain Res
|
1998
|
0.85
|
10
|
Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats.
|
Neuroreport
|
2000
|
0.85
|
11
|
Evidence that L-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients.
|
Cell Mol Neurobiol
|
2010
|
0.85
|
12
|
Effect of chemically induced propionic acidemia on neurobehavioral development of rats.
|
Pharmacol Biochem Behav
|
1999
|
0.84
|
13
|
Inhibition of mitochondrial creatine kinase activity from rat cerebral cortex by methylmalonic acid.
|
Neurochem Int
|
2004
|
0.84
|
14
|
3-hydroxyglutaric acid enhances glutamate uptake into astrocytes from cerebral cortex of young rats.
|
Neurochem Int
|
2004
|
0.83
|
15
|
Effects of methylmalonic and propionic acids on glutamate uptake by synaptosomes and synaptic vesicles and on glutamate release by synaptosomes from cerebral cortex of rats.
|
Brain Res
|
2001
|
0.82
|
16
|
Detection of metabolic disorders in high-risk patients. A pilot study in Salvador, Bahia.
|
Arq Neuropsiquiatr
|
1997
|
0.82
|
17
|
Intrastriatal methylmalonic acid administration induces rotational behavior and convulsions through glutamatergic mechanisms.
|
Brain Res
|
1996
|
0.82
|
18
|
Na+, K+ ATPase activity is markedly reduced by cis-4-decenoic acid in synaptic plasma membranes from cerebral cortex of rats.
|
Exp Neurol
|
2005
|
0.82
|
19
|
Inhibition of in vitro CO2 production and lipid synthesis by 2-hydroxybutyric acid in rat brain.
|
Braz J Med Biol Res
|
2001
|
0.82
|
20
|
[Application of a clinical and laboratory protocol for the investigation of inborn errors of metabolism among critically ill children]
|
J Pediatr (Rio J)
|
2003
|
0.82
|
21
|
Intrastriatal administration of 3-hydroxyglutaric acid induces convulsions and striatal lesions in rats.
|
Brain Res
|
2001
|
0.82
|
22
|
Inhibition of succinate dehydrogenase and beta-hydroxybutyrate dehydrogenase activities by methylmalonate in brain and liver of developing rats.
|
J Inherit Metab Dis
|
1993
|
0.81
|
23
|
Methylmalonate administration decreases Na+,K+-ATPase activity in cerebral cortex of rats.
|
Neuroreport
|
2000
|
0.81
|
24
|
Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease.
|
J Neurol Sci
|
2000
|
0.81
|
25
|
Inhibition of rat brain Na+, K+-ATPase activity induced by homocysteine is probably mediated by oxidative stress.
|
Neurochem Res
|
2001
|
0.81
|
26
|
In vitro inhibition of Na+,K(+)-ATPase activity from rat cerebral cortex by guanidino compounds accumulating in hyperargininemia.
|
Brain Res
|
1999
|
0.81
|
27
|
Alanine reverses the inhibitory effect of phenylalanine and its metabolites on Na+,K(+)-ATPase in synaptic plasma membranes from cerebral cortex of rats.
|
Biochem Soc Trans
|
1995
|
0.80
|
28
|
Inhibition of mitogen and allogeneic stimulated lymphocyte growth by human amniotic fluid: lack of correlation between alpha-fetoprotein level and in vitro immunosuppression.
|
Eur J Obstet Gynecol Reprod Biol
|
1986
|
0.80
|
29
|
3-Hydroxyglutaric acid moderately impairs energy metabolism in brain of young rats.
|
Neuroscience
|
2005
|
0.80
|
30
|
Effect of hyperprolinemia on acetylcholinesterase and butyrylcholinesterase activities in rat.
|
Amino Acids
|
2005
|
0.80
|
31
|
D-2-hydroxyglutaric acid inhibits creatine kinase activity from cardiac and skeletal muscle of young rats.
|
Eur J Clin Invest
|
2003
|
0.80
|
32
|
Neurochemical effects of L-pyroglutamic acid.
|
Neurochem Res
|
1995
|
0.80
|
33
|
Promotion of oxidative stress by 3-hydroxyglutaric acid in rat striatum.
|
J Inherit Metab Dis
|
2005
|
0.80
|
34
|
Animal models for glutaryl-CoA dehydrogenase deficiency.
|
J Inherit Metab Dis
|
2004
|
0.79
|
35
|
Inhibition of energy metabolism by 2-methylacetoacetate and 2-methyl-3-hydroxybutyrate in cerebral cortex of developing rats.
|
J Inherit Metab Dis
|
2005
|
0.79
|
36
|
L-pyroglutamic acid inhibits energy production and lipid synthesis in cerebral cortex of young rats in vitro.
|
Neurochem Res
|
2001
|
0.79
|
37
|
Quinolinic acid inhibits glutamate uptake into synaptic vesicles from rat brain.
|
Neuroreport
|
2000
|
0.79
|
38
|
Nitric oxide synthase inhibition by L-NAME prevents the decrease of Na+,K+-ATPase activity in midbrain of rats subjected to arginine administration.
|
Neurochem Res
|
2001
|
0.79
|
39
|
Reduced locomotor activity of rats made histidinemic by injection of histidine.
|
J Nutr
|
1989
|
0.79
|
40
|
Inhibition of energy production in vitro by glutaric acid in cerebral cortex of young rats.
|
Metab Brain Dis
|
2000
|
0.79
|
41
|
Ascorbic acid and alpha-tocopherol attenuate methylmalonic acid-induced convulsions.
|
Neuroreport
|
1999
|
0.79
|
42
|
Effects of methylmalonate and propionate on uptake of glucose and ketone bodies in vitro by brain of developing rats.
|
Biochem Med Metab Biol
|
1991
|
0.78
|
43
|
The influence of amino acids on mitogen-activated proliferation of human lymphocytes in vitro.
|
Int J Immunopharmacol
|
1994
|
0.78
|
44
|
In vitro stimulation of oxidative stress in cerebral cortex of rats by the guanidino compounds accumulating in hyperargininemia.
|
Brain Res
|
2001
|
0.78
|
45
|
Inhibition of lymphocyte transformation by serum from pregnant women: lack of correlation with levels of alpha-fetoprotein.
|
Biol Neonate
|
1983
|
0.78
|
46
|
Inhibition of in-vitro lymphocyte transformation by the isoquinoline alkaloid berberine.
|
J Pharm Pharmacol
|
1995
|
0.78
|
47
|
Seven-year experience of a reference laboratory for detection of inborn errors of metabolism in Brazil.
|
J Inherit Metab Dis
|
1991
|
0.78
|
48
|
Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats.
|
Brain Res
|
2001
|
0.78
|
49
|
Creatine protects against the convulsive behavior and lactate production elicited by the intrastriatal injection of methylmalonate.
|
Neuroscience
|
2003
|
0.77
|
50
|
Inhibition of rat brain lipid synthesis in vitro by 4-hydroxybutyric acid.
|
Metab Brain Dis
|
1999
|
0.77
|
51
|
Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in rat cerebral cortex and liver.
|
Free Radic Res
|
2013
|
0.77
|
52
|
The influence of organic acids on the proliferation of human peripheral lymphocytes activated by concanavalin A and pokeweed mitogen.
|
Int J Immunopharmacol
|
1996
|
0.77
|
53
|
Oxidative stress in phenylketonuric patients.
|
Mol Genet Metab
|
2011
|
0.77
|
54
|
Platelet Na+, K+-ATPase activity as a possible peripheral marker for the neurotoxic effects of phenylalanine in phenylketonuria.
|
Metab Brain Dis
|
2000
|
0.77
|
55
|
Inhibition of citrate oxidation in vitro by 2-hydroxybutyrate and 4-hydroxybutyrate in cerebral cortex of young rats.
|
Biochem Soc Trans
|
1995
|
0.77
|
56
|
Effects of histidine and imidazolelactic acid on various parameters of the oxidative stress in cerebral cortex of young rats.
|
Int J Dev Neurosci
|
2004
|
0.76
|
57
|
Isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency in a child with metabolic stroke.
|
J Inherit Metab Dis
|
2006
|
0.76
|
58
|
2-Hydroxybutyrate and 4-hydroxybutyrate inhibit CO2 formation from labeled substrates by rat cerebral cortex.
|
Biochem Soc Trans
|
1995
|
0.76
|
59
|
Clinical and biochemical effects of long-term vitamin A administration to a patient with Hurler-Scheie compound.
|
Braz J Med Biol Res
|
1984
|
0.76
|
60
|
Reduction of plasma concentrations of large neutral amino acids in patients with maple syrup urine disease during crises.
|
Arch Dis Child
|
1999
|
0.76
|
61
|
Inhibition of Na+,K+-ATPase from rat brain cortex by propionic acid.
|
Neuroreport
|
1998
|
0.76
|
62
|
Inhibition of synaptosomal [3H]glutamate uptake and [3H]glutamate binding to plasma membranes from brain of young rats by glutaric acid in vitro.
|
J Neurol Sci
|
2000
|
0.76
|
63
|
Investigation of oxidative stress parameters in treated phenylketonuric patients.
|
Metab Brain Dis
|
2006
|
0.76
|
64
|
Chronic early leucine administration induces behavioral deficits in rats.
|
Life Sci
|
1999
|
0.75
|
65
|
Response of human peripheral blood lymphocytes in the presence of cord sera: relationship of lymphocyte transformation with number of pregnancies and levels of alpha-fetoprotein.
|
Clin Exp Immunol
|
1983
|
0.75
|
66
|
Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies.
|
J Inherit Metab Dis
|
2007
|
0.75
|
67
|
An improved specific laboratory test for homocystinuria.
|
Clin Chim Acta
|
1982
|
0.75
|
68
|
Proline administration decreases Na+,K+-ATPase activity in the synaptic plasma membrane from cerebral cortex of rats.
|
Metab Brain Dis
|
1999
|
0.75
|
69
|
Reduced Na(+), K(+)-ATPase activity in erythrocyte membranes from patients with phenylketonuria.
|
Pediatr Res
|
2001
|
0.75
|
70
|
Detection of inborn errors of metabolism in unselected patients from pediatric intensive care units in Porto Alegre, Brazil: evaluation of screening techniques.
|
Braz J Med Biol Res
|
1987
|
0.75
|
71
|
Effects of acute and chronic administration of methylmalonic and propionic acids on the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats.
|
Neurochem Int
|
1998
|
0.75
|
72
|
Creatine prevents behavioral alterations caused by methylmalonic acid administration into the hippocampus of rats in the open field task.
|
J Neurol Sci
|
2006
|
0.75
|
73
|
Influence of methylmalonate on the uptake of ketone bodies in vitro by the brain of young rats.
|
Biochem Soc Trans
|
1990
|
0.75
|
74
|
Inhibition of phytohaemagglutinin-induced lymphocyte blastogenesis by serum from pregnant women: correlation between cortisol level and in vitro immunosuppression.
|
Acta Endocrinol (Copenh)
|
1985
|
0.75
|
75
|
Effect of phenylalanine and its metabolites on ATP diphosphohydrolase activity in synaptosomes from rat cerebral cortex.
|
Neurochem Res
|
1994
|
0.75
|
76
|
Inhibition of Na+,K+-ATPase activity from rat hippocampus by proline.
|
Neurochem Res
|
2001
|
0.75
|
77
|
Effects of methylmalonate and propionate on [3H]glutamate binding, adenylate cyclase activity and lipid synthesis in rat cerebral cortex.
|
Biochem Mol Biol Int
|
1997
|
0.75
|
78
|
Relationship between some obstetric landmarks and the concentration of alpha-fetoprotein in maternal blood.
|
J Perinat Med
|
1986
|
0.75
|
79
|
The influence of aldosterone on the phytohemagglutinin-induced human lymphocyte blastogenesis.
|
Rev Bras Biol
|
1985
|
0.75
|
80
|
Tay-Sachs disease heterozygote detection in Brazil: comparison between tears and leukocytes as beta-hexosaminidase A source.
|
J Inherit Metab Dis
|
1984
|
0.75
|
81
|
The effect of organic acids on phytohaemagglutinin-activated proliferation of human lymphocytes in vitro.
|
Int J Immunopharmacol
|
1995
|
0.75
|
82
|
Alanine prevents the decrease of Na+,K+-ATPase activity in experimental phenylketonuria.
|
Metab Brain Dis
|
1999
|
0.75
|
83
|
Effect of propionic and methylmalonic acids on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex.
|
Neurochem Res
|
2002
|
0.75
|
84
|
Methylmalonic acid reduces the in vitro phosphorylation of cytoskeletal proteins in the cerebral cortex of rats.
|
Brain Res
|
1997
|
0.75
|
85
|
In vitro phosphorylation of cytoskeletal proteins in the rat cerebral cortex is decreased by propionic acid.
|
Exp Neurol
|
1997
|
0.75
|
86
|
Effect of postnatal methylmalonate administration on adult rat behavior.
|
Braz J Med Biol Res
|
1991
|
0.75
|
87
|
Effects of postnatal methylmalonate administration on neurobehavioral development of rats.
|
Braz J Med Biol Res
|
1994
|
0.75
|
88
|
Propionic and methylmalonic acids inhibit the in vitro phosphorylation of a 85 kDa cytoskeletal protein from cerebral cortex of rats.
|
Neurochem Int
|
1998
|
0.75
|
89
|
Alpha-ketoisocaproate increases the in vitro 32P incorporation into intermediate filaments in cerebral cortex of rats.
|
Neuroreport
|
2000
|
0.75
|
90
|
Chronic administration of propionic acid reduces ganglioside N-acetylneuraminic acid concentration in cerebellum of young rats.
|
J Neurol Sci
|
1998
|
0.75
|
91
|
Methylmalonic and propionic acids increase the in vitro incorporation of 32P into cytoskeletal proteins from cerebral cortex of young rats through NMDA glutamate receptors.
|
Brain Res
|
2000
|
0.75
|
92
|
Disruption of redox homeostasis and brain damage caused in vivo by methylmalonic acid and ammonia in cerebral cortex and striatum of developing rats.
|
Free Radic Res
|
2014
|
0.75
|
93
|
Immunomodulatory action of amino acids on lymphocyte blastogenesis.
|
Biochem Soc Trans
|
1992
|
0.75
|
94
|
Effect of the branched-chain alpha-ketoacids accumulating in maple syrup urine disease on the high molecular weight neurofilament subunit (NF-H) in rat cerebral cortex.
|
Metab Brain Dis
|
2002
|
0.75
|
95
|
Effect of phenylalanine, p-chlorophenylalanine and alpha-methylphenylalanine on glucose uptake in vitro by the brain of young rats.
|
Biochem Soc Trans
|
1990
|
0.75
|
96
|
Chronic postnatal administration of methylmalonic acid provokes a decrease of myelin content and ganglioside N-acetylneuraminic acid concentration in cerebrum of young rats.
|
Braz J Med Biol Res
|
2001
|
0.75
|
97
|
Incidence of 3-hydroxy-3-methylglutaryl-coenzyme A lyase (HL) deficiency in Brazil, South America.
|
J Inherit Metab Dis
|
2007
|
0.75
|
98
|
ATP diphosphohydrolase activity in synaptosomes from cerebral cortex of rats subjected to chemically induced phenylketonuria.
|
Braz J Med Biol Res
|
1995
|
0.75
|
99
|
Effect of proline administration on rat behavior in aversive and nonaversive tasks.
|
Pharmacol Biochem Behav
|
1989
|
0.75
|
100
|
Inhibition of glutamate uptake into synaptic vesicles from rat brain by 3-nitropropionic acid in vitro.
|
Exp Neurol
|
2001
|
0.75
|