PubRank

Haruaki Ninomiya

Author PubWeight™ 49.63‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Depletion of Uric Acid Due to SLC22A12 (URAT1) Loss-of-Function Mutation Causes Endothelial Dysfunction in Hypouricemia. Circ J 2015 1.55
2 Electrophysiological properties of prion-positive cardiac progenitors derived from murine embryonic stem cells. Circ J 2012 1.40
3 Mental retardation, spasticity, basal ganglia calcification, cerebral white matter lesions, multiple endocrine defects, telangiectasia and atrophic skin: a new syndrome? Brain Dev 2007 1.39
4 Characterization of sequential N-cadherin cleavage by ADAM10 and PS1. Neurosci Lett 2006 1.22
5 Enhanced autophagy and mitochondrial aberrations in murine G(M1)-gangliosidosis. Biochem Biophys Res Commun 2008 1.21
6 Uricosuric action of losartan via the inhibition of urate transporter 1 (URAT 1) in hypertensive patients. Am J Hypertens 2008 1.15
7 Ubiquitin-proteasome system impairment caused by a missense cardiac myosin-binding protein C mutation and associated with cardiac dysfunction in hypertrophic cardiomyopathy. J Mol Biol 2008 1.15
8 N-octyl-beta-valienamine up-regulates activity of F213I mutant beta-glucosidase in cultured cells: a potential chemical chaperone therapy for Gaucher disease. Biochim Biophys Acta 2004 1.05
9 Endosomal accumulation of Toll-like receptor 4 causes constitutive secretion of cytokines and activation of signal transducers and activators of transcription in Niemann-Pick disease type C (NPC) fibroblasts: a potential basis for glial cell activation in the NPC brain. J Neurosci 2007 0.98
10 The Wilson disease protein ATP7B resides in the late endosomes with Rab7 and the Niemann-Pick C1 protein. Am J Pathol 2005 0.95
11 Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin. Chembiochem 2009 0.94
12 Reciprocal control of hERG stability by Hsp70 and Hsc70 with implication for restoration of LQT2 mutant stability. Circ Res 2010 0.92
13 Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C. Exp Neurol 2004 0.92
14 Activity-dependent regulation of beta-catenin via epsilon-cleavage of N-cadherin. Biochem Biophys Res Commun 2006 0.92
15 Vascular endothelial cells that express dystroglycan are involved in angiogenesis. J Cell Sci 2002 0.92
16 Biotinylated theta-toxin derivative as a probe to examine intracellular cholesterol-rich domains in normal and Niemann-Pick type C1 cells. J Lipid Res 2003 0.91
17 Brainstem neuropathology in a mouse model of Niemann-Pick disease type C. J Neurol Sci 2008 0.90
18 Presenilin 1 is involved in the maturation of beta-site amyloid precursor protein-cleaving enzyme 1 (BACE1). J Neurosci Res 2007 0.90
19 Enzyme enhancement activity of N-octyl-beta-valienamine on beta-glucosidase mutants associated with Gaucher disease. Biochim Biophys Acta 2007 0.89
20 Impairment of ubiquitin-proteasome system by E334K cMyBPC modifies channel proteins, leading to electrophysiological dysfunction. J Mol Biol 2011 0.87
21 Smad expression of hepatic stellate cells in liver cirrhosis in vivo and hepatic stellate cell line in vitro. Pathol Int 2003 0.87
22 A Fluorescent sp2-iminosugar with pharmacological chaperone activity for gaucher disease: synthesis and intracellular distribution studies. Chembiochem 2010 0.86
23 Cholesterol depletion facilitates ubiquitylation of NPC1 and its association with SKD1/Vps4. J Cell Sci 2006 0.86
24 Chemical chaperone therapy: chaperone effect on mutant enzyme and cellular pathophysiology in β-galactosidase deficiency. Hum Mutat 2011 0.85
25 Hsp90 prevents interaction between CHIP and HERG proteins to facilitate maturation of wild-type and mutant HERG proteins. Cardiovasc Res 2013 0.85
26 Subtype switching of T-type Ca 2+ channels from Cav3.2 to Cav3.1 during differentiation of embryonic stem cells to cardiac cell lineage. Circ J 2005 0.84
27 Evidence for proteasomal degradation of Kv1.5 channel protein. Biochem Biophys Res Commun 2005 0.84
28 New GAA mutations in Japanese patients with GSDII (Pompe disease). Pediatr Neurol 2003 0.83
29 Novel TSC1 and TSC2 mutations in Japanese patients with tuberous sclerosis complex. Brain Dev 2002 0.83
30 Reduced sensitivity of Niemann-Pick C1-deficient cells to theta-toxin (perfringolysin O): sequestration of toxin to raft-enriched membrane vesicles. Histochem Cell Biol 2004 0.83
31 Niemann-Pick type C disease: novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency. Mol Genet Metab 2005 0.82
32 Genotype/phenotype of 6 Chinese cases with Niemann-Pick disease type C. Gene 2012 0.82
33 Niemann-Pick C1 protein transports copper to the secretory compartment from late endosomes where ATP7B resides. Exp Cell Res 2008 0.81
34 Stabilizing effects of eicosapentaenoic acid on Kv1.5 channel protein expressed in mammalian cells. Eur J Pharmacol 2008 0.81
35 Copper incorporation into ceruloplasmin is regulated by Niemann-Pick C1 protein. Hepatol Res 2011 0.81
36 The TSC1 gene product hamartin interacts with NADE. Mol Cell Neurosci 2007 0.81
37 Adipose stem cell sheets improved cardiac function in the rat myocardial infarction, but did not alter cardiac contractile responses to β-adrenergic stimulation. Biomed Res 2015 0.80
38 Proteasomal degradation of Kir6.2 channel protein and its inhibition by a Na+ channel blocker aprindine. Biochem Biophys Res Commun 2005 0.79
39 Structural basis of the GM2 gangliosidosis B variant. J Hum Genet 2003 0.79
40 Lysosomal accumulation of Trk protein in brain of GM₁ -gangliosidosis mouse and its restoration by chemical chaperone. J Neurochem 2011 0.79
41 Effect of losartan and benzbromarone on the level of human urate transporter 1 mRNA. Arzneimittelforschung 2010 0.79
42 A bicyclic 1-deoxygalactonojirimycin derivative as a novel pharmacological chaperone for GM1 gangliosidosis. Mol Ther 2013 0.79
43 Stabilization of Kv1.5 channel protein by bepridil through its action as a chemical chaperone. Eur J Pharmacol 2012 0.78
44 Remote reperfusion lung injury is associated with AMP deaminase 3 activation and attenuated by inosine monophosphate. Circ J 2007 0.78
45 Changes of HCN gene expression and I(f) currents in Nkx2.5-positive cardiomyocytes derived from murine embryonic stem cells during differentiation. Biomed Res 2008 0.78
46 Different distribution of Cav3.2 and Cav3.1 transcripts encoding T-type Ca(2+) channels in the embryonic heart of mice. Biomed Res 2010 0.78
47 Effects of azelnidipine on uric acid metabolism in patients with essential hypertension. Clin Exp Hypertens 2014 0.78
48 Molecular basis of 1-deoxygalactonojirimycin arylthiourea binding to human α-galactosidase a: pharmacological chaperoning efficacy on Fabry disease mutants. ACS Chem Biol 2014 0.77
49 Functional stabilization of Kv1.5 protein by Hsp70 in mammalian cell lines. Biochem Biophys Res Commun 2008 0.77
50 Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus in a mouse model of Niemann-Pick disease type C. Brain Res 2004 0.77
51 Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients. Brain Dev 2004 0.77
52 Effects of cilnidipine on serum uric acid level and urinary nitrogen monoxide excretion in patients with hypertension. Clin Exp Hypertens 2012 0.77
53 Inhibition of inward rectifier K+ currents by angiotensin II in rat atrial myocytes: lack of effects in cells from spontaneously hypertensive rats. Hypertens Res 2006 0.77
54 Molecular Mechanisms Underlying Urate-Induced Enhancement of Kv1.5 Channel Expression in HL-1 Atrial Myocytes. Circ J 2015 0.76
55 Plasma levels of uridine correlate with blood pressure and indicators of myogenic purine degradation and insulin resistance in hypertensive patients. Circ J 2007 0.76
56 Antisense suppression of TSC1 gene product, hamartin, enhances neurite outgrowth in NGF-treated PC12h cells. Brain Dev 2007 0.76
57 AMP deaminase 3 plays a critical role in remote reperfusion lung injury. Biochem Biophys Res Commun 2013 0.76
58 Effects of angiotensin II on the action potential durations of atrial myocytes in hypertensive rats. Hypertens Res 2005 0.76
59 Apoptosis induced by an uromodulin mutant C112Y and its suppression by topiroxostat. Clin Exp Nephrol 2014 0.75
60 Candidate molecules for chemical chaperone therapy of GM1-gangliosidosis. Future Med Chem 2013 0.75
61 Chemical chaperone therapy: luciferase assay for screening of β-galactosidase mutations. Mol Genet Metab 2010 0.75
62 The effect of N-octyl-β-valienamine on β-glucosidase activity in tissues of normal mice. Brain Dev 2010 0.75
63 Effects of a low-dose antihypertensive diuretic in combination with losartan, telmisartan, or candesartan on serum urate levels in hypertensive patients. Arzneimittelforschung 2010 0.75
64 The pharmacological chaperone effect of N-octyl-beta-valienamine on human mutant acid beta-glucosidases. Blood Cells Mol Dis 2009 0.75
65 Instability of KCNE1-D85N that causes long QT syndrome: stabilization by verapamil. Pacing Clin Electrophysiol 2014 0.75
66 A case of variant biochemical phenotype of Niemann-Pick disease type C accompanying savant syndrome. Rinsho Shinkeigaku 2016 0.75
67 Novel TSC2 mutations and decreased expression of tuberin in cultured tumor cells with an insertion mutation. Hum Mutat 2004 0.75
68 M3 Muscarinic Receptor Signaling Stabilizes a Novel Mutant Human Ether-a-Go-Go-Related Gene Channel Protein via Phosphorylation of Heat Shock Factor 1 in Transfected Cells. Circ J 2016 0.75
69 Delayed onset of beating and decreased expression of T-type Ca2+ channel in mouse ES cell-derived cardiocytes carrying human chromosome 21. Biochem Biophys Res Commun 2006 0.75
70 1H, 13C and 15N assignments of the tandem WW domains of human MAGI-1/BAP-1. J Biomol NMR 2004 0.75
71 Novel effects of extracts from poisonous mushrooms on expression and function of the human ether-a-go-go-related gene channel. Biol Pharm Bull 2011 0.75