Published in Neurobiol Dis on May 22, 2008
Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms. Acta Neuropathol (2013) 2.30
Lipodystrophies: disorders of adipose tissue biology. Biochim Biophys Acta (2009) 1.63
Lack of testicular seipin causes teratozoospermia syndrome in men. Proc Natl Acad Sci U S A (2014) 1.49
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. J Biol Chem (2009) 1.44
Molecular characterization of seipin and its mutants: implications for seipin in triacylglycerol synthesis. J Lipid Res (2011) 1.14
Seipin: from human disease to molecular mechanism. J Lipid Res (2012) 1.12
The human lipodystrophy protein seipin is an ER membrane adaptor for the adipogenic PA phosphatase lipin 1. Mol Metab (2012) 1.00
Seipin oligomers can interact directly with AGPAT2 and lipin 1, physically scaffolding critical regulators of adipogenesis. Mol Metab (2015) 0.89
Analysis of naturally occurring mutations in the human lipodystrophy protein seipin reveals multiple potential pathogenic mechanisms. Diabetologia (2013) 0.88
Alternative splicing of Spg7, a gene involved in hereditary spastic paraplegia, encodes a variant of paraplegin targeted to the endoplasmic reticulum. PLoS One (2012) 0.86
Re-analysis of an original CMTX3 family using exome sequencing identifies a known BSCL2 mutation. Muscle Nerve (2013) 0.85
Neuroanatomical characterisation of the expression of the lipodystrophy and motor-neuropathy gene Bscl2 in adult mouse brain. PLoS One (2012) 0.78
A Patient with Congenital Generalized Lipodystrophy Due To a Novel Mutation in BSCL2: Indications for Secondary Mitochondrial Dysfunction. JIMD Rep (2011) 0.78
Segregated responses of mammary gland development and vaginal opening to prepubertal genistein exposure in Bscl2(-/-) female mice with lipodystrophy. Reprod Toxicol (2014) 0.77
Seipin knockout in mice impairs stem cell proliferation and progenitor cell differentiation in the adult hippocampal dentate gyrus via reduced levels of PPARγ. Dis Model Mech (2015) 0.77
Motor neuron degeneration in a mouse model of seipinopathy. Cell Death Dis (2013) 0.76
Seipin mutation at glycosylation sites activates autophagy in transfected cells via abnormal large lipid droplets generation. Acta Pharmacol Sin (2015) 0.75
Whole Exome Sequencing Reveals a BSCL2 Mutation Causing Progressive Encephalopathy with Lipodystrophy (PELD) in an Iranian Pediatric Patient. Iran Biomed J (2016) 0.75
Neuronal seipin knockout facilitates Aβ-induced neuroinflammation and neurotoxicity via reduction of PPARγ in hippocampus of mouse. J Neuroinflammation (2016) 0.75
Autophagy as an Emerging Common Pathomechanism in Inherited Peripheral Neuropathies. Front Mol Neurosci (2017) 0.75
Autoimmune targets of heart and skeletal muscles in myasthenia gravis. Arch Neurol (2009) 2.42
High-sensitivity C-reactive protein is an independent clinical feature of nonalcoholic steatohepatitis (NASH) and also of the severity of fibrosis in NASH. J Gastroenterol (2007) 2.29
Modeling familial Alzheimer's disease with induced pluripotent stem cells. Hum Mol Genet (2011) 2.26
Clinical factors predictive of spontaneous remission or relapse in cases of autoimmune pancreatitis. Gastrointest Endosc (2007) 2.23
Mitochondrial dysfunction associated with increased oxidative stress and α-synuclein accumulation in PARK2 iPSC-derived neurons and postmortem brain tissue. Mol Brain (2012) 2.18
Metformin suppresses colorectal aberrant crypt foci in a short-term clinical trial. Cancer Prev Res (Phila) (2010) 1.86
Prevention of cardiovascular events with calcium channel blocker-based combination therapies in patients with hypertension: a randomized controlled trial. J Hypertens (2011) 1.75
Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS. Ann Neurol (2010) 1.71
The MG-QOL15 Japanese version: validation and associations with clinical factors. Muscle Nerve (2012) 1.65
Inositol hexakisphosphate kinase-2, a physiologic mediator of cell death. J Biol Chem (2004) 1.63
Ambivalent aspects of interleukin-6 in cerebral ischemia: inflammatory versus neurotrophic aspects. J Cereb Blood Flow Metab (2008) 1.59
Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS. Neurology (2011) 1.55
Intracardiac calipers for artificial chordae replacement in mitral valve repair. Ann Thorac Surg (2009) 1.55
Contribution of the flow effect caused by shear-dependent RBC aggregation to NIR spectroscopic signals. Neuroimage (2006) 1.52
Parkin-catalyzed ubiquitin-ester transfer is triggered by PINK1-dependent phosphorylation. J Biol Chem (2013) 1.50
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. J Biol Chem (2009) 1.44
Evidence against strong correlation between chest symptoms and ischemic coronary changes after subcutaneous sumatriptan injection. Intern Med (2002) 1.40
Blockade of interleukin-6 signaling aggravates ischemic cerebral damage in mice: possible involvement of Stat3 activation in the protection of neurons. J Neurochem (2005) 1.34
Molecular pathogenesis of seipin/BSCL2-related motor neuron diseases. Ann Neurol (2007) 1.28
Esophageal cancer-related gene 4 is a secreted inducer of cell senescence expressed by aged CNS precursor cells. Proc Natl Acad Sci U S A (2010) 1.24
Seipinopathy: a novel endoplasmic reticulum stress-associated disease. Brain (2008) 1.22
Slowly progressive spread of the stroke-like lesions in MELAS. Neurology (2003) 1.21
Human DDX3Y, the Y-encoded isoform of RNA helicase DDX3, rescues a hamster temperature-sensitive ET24 mutant cell line with a DDX3X mutation. Exp Cell Res (2004) 1.21
Characterization of the mitochondrial protein LETM1, which maintains the mitochondrial tubular shapes and interacts with the AAA-ATPase BCS1L. J Cell Sci (2008) 1.21
Abnormal expression of TIP30 and arrested nucleocytoplasmic transport within oligodendrocyte precursor cells in multiple sclerosis. J Clin Invest (2008) 1.19
Regeneration of the central nervous system using endogenous repair mechanisms. J Neurochem (2007) 1.17
Comparative Study of 2 Different Questionnaires in Japanese Patients: The Quality of Life and Utility Evaluation Survey Technology Questionnaire (QUEST) Versus the Frequency Scale for the Symptoms of Gastroesophageal Reflux Disease Questionnaire (FSSG). J Neurogastroenterol Motil (2013) 1.17
Notch3 ectodomain is a major component of granular osmiophilic material (GOM) in CADASIL. Acta Neuropathol (2006) 1.12
Control of brain capillary blood flow. J Cereb Blood Flow Metab (2012) 1.12
Identification of Cystatin SN as a novel tumor marker for colorectal cancer. Int J Oncol (2009) 1.09
Evaporation-induced coating of hydrous ruthenium oxide on mesoporous silica nanoparticles to develop high-performance supercapacitors. Small (2013) 1.07
Determination of editors at the novel A-to-I editing positions. Neurosci Res (2008) 1.06
Resident endothelial cells surrounding damaged arterial endothelium reendothelialize the lesion. Arterioscler Thromb Vasc Biol (2010) 1.06
Current concepts in multiple sclerosis: autoimmunity versus oligodendrogliopathy. Clin Rev Allergy Immunol (2012) 1.05
Rectal fecaloma: successful treatment using endoscopic removal. Digestion (2007) 1.04
Association between angiotensin II type 1 receptor polymorphisms and the occurrence of nonalcoholic fatty liver disease. Liver Int (2009) 1.04
Solitary Peutz-Jeghers type hamartomatous polyps in the duodenum are not always associated with a low risk of cancer: two case reports. J Med Case Rep (2011) 1.03
O-glycosylation of mucin-like domain retains the neutral ceramidase on the plasma membranes as a type II integral membrane protein. J Biol Chem (2002) 1.02
Oxidative metabolism in cultured rat astroglia: effects of reducing the glucose concentration in the culture medium and of D-aspartate or potassium stimulation. J Cereb Blood Flow Metab (2006) 1.02
Accelerating progress in induced pluripotent stem cell research for neurological diseases. Ann Neurol (2012) 1.01
Health-related quality-of-life and treatment targets in myasthenia gravis. Muscle Nerve (2014) 1.01
Statins and myotoxic effects associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies: an observational study in Japan. Medicine (Baltimore) (2015) 1.01
N88S seipin mutant transgenic mice develop features of seipinopathy/BSCL2-related motor neuron disease via endoplasmic reticulum stress. Hum Mol Genet (2011) 1.01
Efficacy of Lactobacillus casei treatment on small bowel injury in chronic low-dose aspirin users: a pilot randomized controlled study. J Gastroenterol (2011) 1.00
Plasma Pentraxin3 is a novel marker for nonalcoholic steatohepatitis (NASH). BMC Gastroenterol (2008) 0.99
Overlooked non-motor symptoms in myasthenia gravis. J Neurol Neurosurg Psychiatry (2012) 0.99
Enhanced aggregation of androgen receptor in induced pluripotent stem cell-derived neurons from spinal and bulbar muscular atrophy. J Biol Chem (2013) 0.99
Factors predicting the presence of small bowel lesions in patients with obscure gastrointestinal bleeding. Dig Endosc (2012) 0.98
Alcohol consumption is associated with an increased risk of erosive esophagitis and Barrett's epithelium in Japanese men. BMC Gastroenterol (2008) 0.98
The combination therapy of hypertension to prevent cardiovascular events (COPE) trial: rationale and design. Hypertens Res (2005) 0.98
Oscillating neuro-capillary coupling during cortical spreading depression as observed by tracking of FITC-labeled RBCs in single capillaries. Neuroimage (2011) 0.97
Therapeutic effect of IL-12/23 and their signaling pathway blockade on brain ischemia model. Biochem Biophys Res Commun (2010) 0.97
Group A PP2Cs evolved in land plants as key regulators of intrinsic desiccation tolerance. Nat Commun (2013) 0.97
Association between PPARGC1A polymorphisms and the occurrence of nonalcoholic fatty liver disease (NAFLD). BMC Gastroenterol (2008) 0.96
An epidemiologic survey of chronic intestinal pseudo-obstruction and evaluation of the newly proposed diagnostic criteria. Digestion (2012) 0.96
Progressive multifocal leukoencephalopathy developed in incomplete Heerfordt syndrome, a rare manifestation of sarcoidosis, without steroid therapy responding to cidofovir. Clin Neurol Neurosurg (2009) 0.96
Intima-media thickening of the radial artery after transradial intervention. An intravascular ultrasound study. J Am Coll Cardiol (2003) 0.95
Serum ferritin is a clinical biomarker in Japanese patients with nonalcoholic steatohepatitis (NASH) independent of HFE gene mutation. Dig Dis Sci (2009) 0.95
Epidemiological features of moyamoya disease in Japan. Neurol Med Chir (Tokyo) (2012) 0.95
Dually supplied T-junctions in arteriolo-arteriolar anastomosis in mice: key to local hemodynamic homeostasis in normal and ischemic states? Stroke (2009) 0.95
Heterogeneity in expression and subcellular localization of tight junction proteins, claudin-10 and -15, examined by RT-PCR and immunofluorescence microscopy. Arch Histol Cytol (2005) 0.94
Visceral obesity and the risk of Barrett's esophagus in Japanese patients with non-alcoholic fatty liver disease. BMC Gastroenterol (2009) 0.94
Risky endoscopic ultrasonography-guided fine-needle aspiration for asymptomatic retroperitoneal tumors. Dig Endosc (2010) 0.94
Molecular cloning and functional analysis of zebrafish neutral ceramidase. J Biol Chem (2004) 0.94
Astroglial pentose phosphate pathway rates in response to high-glucose environments. ASN Neuro (2012) 0.94
Effects of Histamine-2 Receptor Antagonists and Proton Pump Inhibitors on the Rate of Gastric Emptying: A Crossover Study Using a Continuous Real-Time C Breath Test (BreathID System). J Neurogastroenterol Motil (2011) 0.94
Myopathy associated with antibodies to signal recognition particle: disease progression and neurological outcome. Arch Neurol (2012) 0.94
Camptocormia in Japanese patients with Parkinson's disease: a multicenter study. Mov Disord (2011) 0.94
Expression of adiponectin receptors, AdipoR1 and AdipoR2, in normal colon epithelium and colon cancer tissue. Oncol Rep (2008) 0.92
Novel autoantibodies to a voltage-gated potassium channel Kv1.4 in a severe form of myasthenia gravis. J Neuroimmunol (2005) 0.92
RBC velocities in single capillaries of mouse and rat brains are the same, despite 10-fold difference in body size. Brain Res (2010) 0.92
Assessment of small bowel motility in patients with chronic intestinal pseudo-obstruction using cine-MRI. Am J Gastroenterol (2013) 0.91
The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis. Mol Brain (2013) 0.91
Distribution and origin of TRPV1 receptor-containing nerve fibers in the dura mater of rat. Brain Res (2007) 0.91
Activation of cytokine signaling through leukemia inhibitory factor receptor (LIFR)/gp130 attenuates ischemic brain injury in rats. J Cereb Blood Flow Metab (2005) 0.91
II. Cerebral infarction/transient ischemic attack (TIA). J Stroke Cerebrovasc Dis (2011) 0.91
Early aggressive treatment strategy against myasthenia gravis. Eur Neurol (2010) 0.91
Factors predictive of relapse and spontaneous remission of autoimmune pancreatitis patients treated/not treated with corticosteroids. J Gastroenterol (2011) 0.90
Degeneration of mesencephalic dopaminergic neurons in klotho mouse related to vitamin D exposure. Brain Res (2011) 0.90
Abdominal surgery affects small bowel transit time and completeness of capsule endoscopy. Dig Dis Sci (2008) 0.90
G501C polymorphism of oxidized LDL receptor gene (OLR1) and ischemic stroke. Brain Res (2006) 0.90
Establishment of induced pluripotent stem cells from centenarians for neurodegenerative disease research. PLoS One (2012) 0.90
Does postprandial coffee intake enhance gastric emptying?: a crossover study using continuous real time 13C breath test (BreathID system). Hepatogastroenterology (2009) 0.90
Capillary remodeling and collateral growth without angiogenesis after unilateral common carotid artery occlusion in mice. Microcirculation (2011) 0.90
Reduction of TRPV1 expression in the trigeminal system by botulinum neurotoxin type-A. Neurobiol Dis (2012) 0.89
Colonic metastasis from hepatocellular carcinoma: manifested by gastrointestinal bleeding. Dig Dis Sci (2008) 0.89
Tektin5, a new Tektin family member, is a component of the middle piece of flagella in rat spermatozoa. Mol Reprod Dev (2008) 0.89
Complementary DNA cloning and characterization of rat spergen-1, a spermatogenic cell-specific gene-1, containing a mitochondria-targeting signal. Biol Reprod (2002) 0.89
Nukacin ISK-1, a bacteriostatic lantibiotic. Antimicrob Agents Chemother (2009) 0.89
Ulcerative colitis with Takayasu disease. Digestion (2010) 0.89
Cardiac parasympathetic dysfunction concurrent with cardiac sympathetic denervation in Parkinson's disease. J Neurol Sci (2008) 0.88
Clinical significance of swollen duodenal papilla in autoimmune pancreatitis. Pancreas (2007) 0.88
Three types of striational antibodies in myasthenia gravis. Autoimmune Dis (2011) 0.88
Unique catabolic pathway of glycosphingolipids in a hydrozoan, Hydra magnipapillata, involving endoglycoceramidase. J Biol Chem (2004) 0.88
Inhibition of peroxisome proliferator-activated receptor gamma activity suppresses pancreatic cancer cell motility. Cancer Sci (2008) 0.88
Shape of Barrett's epithelium is associated with prevalence of erosive esophagitis. World J Gastroenterol (2010) 0.88
Expression of Mina53, a product of a Myc target gene in mouse testis. Int J Androl (2006) 0.88
Molecular cloning of a new member of TEKTIN family, Tektin4, located to the flagella of rat spermatozoa. Mol Reprod Dev (2005) 0.88
Electrochemical synthesis of mesoporous Pt-Au binary alloys with tunable compositions for enhancement of electrochemical performance. J Am Chem Soc (2012) 0.87
Effects of body positions on gastric emptying with enteral nutrition: a crossover study using a continuous real time 13C breath test (BreathID system). Hepatogastroenterology (2008) 0.87