Published in Neurology on May 28, 2002
Intermediate filaments: primary determinants of cell architecture and plasticity. J Clin Invest (2009) 1.88
Adult-onset Alexander disease with typical "tadpole" brainstem atrophy and unusual bilateral basal ganglia involvement: a case report and review of the literature. BMC Neurol (2010) 1.07
Propensity for paternal inheritance of de novo mutations in Alexander disease. Hum Genet (2005) 0.97
Intermediate filament proteinopathies: from cytoskeletons to genes to functional nosology. Neurology (2002) 0.75
Acute cerebellitis. Cerebellum (2002) 1.22
Efficient biosynthesis of D-allose from D-psicose by cross-linked recombinant L-rhamnose isomerase: separation of product by ethanol crystallization. J Biosci Bioeng (2006) 1.15
Crystal structures of D-tagatose 3-epimerase from Pseudomonas cichorii and its complexes with D-tagatose and D-fructose. J Mol Biol (2007) 0.93
Recurrent URAT1 gene mutations and prevalence of renal hypouricemia in Japanese. Pediatr Nephrol (2005) 0.92
Prolonged hypothermia protects neonatal rat brain against hypoxic-ischemia by reducing both apoptosis and necrosis. Brain Dev (2005) 0.92
Novel reactions of L-rhamnose isomerase from Pseudomonas stutzeri and its relation with D-xylose isomerase via substrate specificity. Biochim Biophys Acta (2004) 0.92
Hypocretin-1 (orexin-A) levels in human lumbar CSF in different age groups: infants to elderly persons. Sleep (2002) 0.91
The structures of L-rhamnose isomerase from Pseudomonas stutzeri in complexes with L-rhamnose and D-allose provide insights into broad substrate specificity. J Mol Biol (2006) 0.91
Cloning, nucleotide sequence, and overexpression of the L-rhamnose isomerase gene from Pseudomonas stutzeri in Escherichia coli. Appl Environ Microbiol (2004) 0.89
Calpain inhibitor MDL 28170 protects hypoxic-ischemic brain injury in neonatal rats by inhibition of both apoptosis and necrosis. Brain Res (2005) 0.89
Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients. Mol Genet Metab (2004) 0.89
Stroke-like episode involving a cerebral artery in a patient with MELAS. Pediatr Neurol (2005) 0.87
Neuroprotection of edaravone on hypoxic-ischemic brain injury in neonatal rats. Brain Res Dev Brain Res (2004) 0.87
Noninvasive visualization and measurement of posterior descending coronary artery flow velocity by transthoracic Doppler echocardiography in normal children. Am J Cardiol (2003) 0.85
Johanson-blizzard syndrome: loss of glucagon secretion response to insulin-induced hypoglycemia. J Pediatr Endocrinol Metab (2004) 0.84
Serum uric acid as an obesity-related indicator in early adolescence. Tohoku J Exp Med (2006) 0.83
Lidocaine-dependent early infantile status epilepticus with highly suppressed EEG. Epilepsia (2002) 0.82
Elevated sphingomyelinase and hypercytokinemia in hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol (2002) 0.82
Niemann-Pick type C disease: novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency. Mol Genet Metab (2005) 0.82
Pulse methylprednisolone therapy in type 3 adenovirus pneumonia with hypercytokinemia. Tohoku J Exp Med (2006) 0.82
Myocardial acceleration during isovolumic contraction as a new index of right ventricular contractile function and its relation to pulmonary regurgitation in patients after repair of tetralogy of Fallot. J Am Soc Echocardiogr (2004) 0.82
Left anterior descending coronary artery flow and its relation to age in children. J Am Soc Echocardiogr (2002) 0.82
Nationwide survey (incidence, clinical course, prognosis) of Rasmussen's encephalitis. Brain Dev (2009) 0.81
A novel enzymatic approach to the massproduction of L-galactose from L-sorbose. J Biosci Bioeng (2004) 0.81
Niemann-Pick disease type C: cataplexy and hypocretin in cerebrospinal fluid. Tohoku J Exp Med (2006) 0.81
Characterization of the 105-kDa molecular chaperone. Identification, biochemical properties, and localization. Eur J Biochem (2002) 0.81
Novel substrate specificity of D-arabinose isomerase from Klebsiella pneumoniae and its application to production of D-altrose from D-psicose. J Biosci Bioeng (2006) 0.80
Distal pulmonary cell proliferation is associated with the expression of EIIIA+ fibronectin in the developing rat lung. Exp Lung Res (2003) 0.80
Direct production of D-arabinose from D-xylose by a coupling reaction using D-xylose isomerase, D-tagatose 3-epimerase and D-arabinose isomerase. J Biosci Bioeng (2003) 0.80
Increased numbers of macrophages in tracheal aspirates in premature infants with funisitis. Pediatr Int (2008) 0.80
Elastogenesis in cultured dermal fibroblasts from patients with lysosomal beta-galactosidase, protective protein/cathepsin A and neuraminidase-1 deficiencies. J Med Invest (2006) 0.80
A hippocampal lesion detected by high-field 3 tesla magnetic resonance imaging in a patient with temporal lobe epilepsy. Tohoku J Exp Med (2005) 0.79
Crystallization and preliminary X-ray diffraction studies of L-rhamnose isomerase from Pseudomonas stutzeri. Acta Crystallogr Sect F Struct Biol Cryst Commun (2006) 0.79
Assessment of a new piezoelectric transducer sensor for noninvasive cardiorespiratory monitoring of newborn infants in the NICU. Neonatology (2010) 0.79
Case of congenital infection with Candida glabrata in one infant in a set of twins. Pediatr Int (2002) 0.78
Cloning and sequencing of an exoglucanase gene from Streptomyces sp. M 23, and its expression in Streptomyces lividans TK-24. J Biosci Bioeng (2005) 0.78
Acid sphingomyelinase: relation of 93lysine residue on the ratio of intracellular to secreted enzyme activity. Tohoku J Exp Med (2005) 0.78
Renovascular hypertension due to antithrombin deficiency in childhood. Pediatr Nephrol (2004) 0.78
Codevelopment of dendritic cells along with erythroid differentiation from human CD34(+) cells by tumor necrosis factor-alpha. Exp Hematol (2004) 0.78
Paradoxical relationship between B-type natriuretic peptide and pulmonary vascular resistance in patients with ventricular septal defect and concomitant severe pulmonary hypertension. Pediatr Cardiol (2007) 0.77
Blood flow in the left anterior descending coronary artery in children with ventricular septal defect. J Am Soc Echocardiogr (2002) 0.77
A PTPN11 gene mutation (Y63C) causing Noonan syndrome is not associated with short stature in general population. Tohoku J Exp Med (2006) 0.77
Intraventricular ascorbic acid administration decreases hypoxic-ischemic brain injury in newborn rats. Brain Res (2006) 0.77
Significant decrease in tropoelastin gene expression in fibroblasts from a Japanese Costello syndrome patient with impaired elastogenesis and enhanced proliferation. J Biochem (2006) 0.77
Osteopontin expression in the liver with severe perisinusoidal fibrosis: autopsy case of Down syndrome with transient myeloproliferative disorder. Pathol Int (2008) 0.77
Long-acting gonadotropin-releasing hormone analogue treatment for central precocious puberty in maternal uniparental disomy chromosome 14. Tohoku J Exp Med (2005) 0.77
Tropoelastin regulates chemokine expression in fibroblasts in Costello syndrome. Biochem Biophys Res Commun (2008) 0.76
Signaling pathway for radiation-induced apoptosis in the lymphoblasts from neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease. J Neurol Sci (2002) 0.76
A novel bioconversion of L-fructose to L-glucose by Klebsiella pneumoniae. J Biosci Bioeng (2003) 0.76
Elevated Urinary Levels of 8-Hydroxy-2'-deoxyguanosine in a Japanese Child of Xeroderma Pigmentosum/Cockayne Syndrome Complex with Infantile Onset of Nephrotic Syndrome. Tohoku J Exp Med (2016) 0.76
A molecular lesion in a Japanese patient with severe phenotype of 3-methylglutaconic aciduria type I. Pediatr Int (2005) 0.76
Dexamethasone-induced prenatal alveolar wall thinning is associated with a decrease in EIIIA+ fibronectin isoform in the fetal rat lung. Biol Neonate (2004) 0.76
Aseptic meningitis associated with cephalosporins in an infant with trisomy 21. J Child Neurol (2007) 0.75
Luteinizing hormone-releasing hormone analog treatment for a criminal sexual habit caused by male precocious puberty. Pediatr Int (2005) 0.75
Overexpression, purification, crystallization and preliminary X-ray crystal analysis of Bacillus pallidusD-arabinose isomerase. Acta Crystallogr Sect F Struct Biol Cryst Commun (2008) 0.75
Purification, crystallization and preliminary X-ray diffraction studies of D-tagatose 3-epimerase from Pseudomonas cichorii. Acta Crystallogr Sect F Struct Biol Cryst Commun (2007) 0.75
Usefulness of exercise-induced changes in plasma levels of brain natriuretic peptide in predicting right ventricular contractile reserve after repair of tetralogy of Fallot. Am J Cardiol (2005) 0.75
Five novel SLC7A7 variants and y+L gene-expression pattern in cultured lymphoblasts from Japanese patients with lysinuric protein intolerance. Hum Mutat (2002) 0.75
Left ventricular systolic and diastolic function during early neonatal period using transthoracic echocardiography. Tohoku J Exp Med (2002) 0.75
Alexander disease with mild dorsal brainstem atrophy and infantile spasms. Brain Dev (2012) 0.75
Hollow fibers for delivery of harmonic pulses of Q-switched Nd:YAG lasers. Appl Opt (2002) 0.75
Choroid plexus carcinoma presented with spinal dysfunction caused by a drop metastasis: a case report. J Neurooncol (2003) 0.75
Novel mutation of methylmalonyl-CoA mutase gene causing the mut0 form of methylmalonic acidemia in a Japanese girl. Pediatr Int (2007) 0.75
Leukoencephalopathy around a tumor cyst following intracystic methotrexate injection. Pediatr Neurol (2005) 0.75
Split notochord syndrome: ileal duplication causing intermittent episodes of vomiting. Tohoku J Exp Med (2006) 0.75
Familial mediterranean fever medicated with an herbal medicine in Japan. Pediatr Int (2004) 0.75
X-linked adrenal hypoplasia congenita: testicular histology before puberty. Pediatr Int (2007) 0.75
Overexpression of Aspergillus aculeatus cellobiohydrolase I in Aspergillus oryzae. J Biosci Bioeng (2003) 0.75
Correlation between right ventricular Tei index by tissue Doppler imaging and pulsed Doppler imaging in fetuses. Pediatr Cardiol (2008) 0.75
[Symptomatic hypersomnia due to orexin deficiency in hypothalamic lesions]. No To Hattatsu (2006) 0.75
The efficacy of using pulmonary vein wedge pressure for the estimation of pulmonary artery pressure in children with congenital heart disease. Catheter Cardiovasc Interv (2003) 0.75
Normal values for left anterior descending coronary artery flow velocity assessed by transthoracic doppler echocardiography in healthy children. Tohoku J Exp Med (2003) 0.75