A5056345650- Complement system in diseases
- Platelet Disorders and Treatments
- Renal Diseases and Glomerulopathies
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Neuroscience and Neuropharmacology Research
- Amino Acid Enzymes and Metabolism
- Blood groups and transfusion
- Pain Mechanisms and Treatments
- Metabolism and Genetic Disorders
- Neuroinflammation and Neurodegeneration Mechanisms
- Nerve injury and regeneration
- Blood Coagulation and Thrombosis Mechanisms
- Hemoglobinopathies and Related Disorders
- Renal Transplantation Outcomes and Treatments
- Neurogenesis and neuroplasticity mechanisms
- Circadian rhythm and melatonin
- Liver Disease and Transplantation
- Blood donation and transfusion practices
- Neuroendocrine regulation and behavior
- Exercise and Physiological Responses
- Neurological disorders and treatments
- Iron Metabolism and Disorders
- Botulinum Toxin and Related Neurological Disorders
- Kawasaki Disease and Coronary Complications
- Extracellular vesicles in disease
Nara Medical University
2016-2025
Nara Medical University Hospital
2012-2024
Kaketsuken (Japan)
2013
Fujita Health University
2010
Japan Research Institute
2006
Murata (United States)
2004
BACKGROUND: ADAMTS13 specifically cleaves unusually large von Willebrand factor (VWF) multimers, which induce platelet thrombi formation under high shear stress. activity is deficient in patients with thrombotic thrombocytopenic purpura (TTP). The determination of plasma levels a prerequisite for differential diagnosis microangiopathies. Here, unique and highly sensitive enzyme immunoassay (EIA) described. STUDY DESIGN AND METHODS: hydrolyzes the peptide bond between Y1605 M1606 VWF. In this...
Summary Decreased plasma ADAMTS13 activity (ADAMTS13:AC) results in the accumulation of unusually large von Willebrand factor multimer (UL-VWFM) and formation platelet thrombi. It remains controversial whether or not ADAMTS13:AC decreases patients with liver cirrhosis (LC), its relationship to clinical features has been fully investigated. We measured related parameters 33 chronic hepatitis (CH) 109 LC. decreased increasing severity disease (controls means 100%, CH 87%, Child A-LC 79%, B-LC...
Astrocytes are the most abundant glia cell type in central nervous system (CNS), and known to constitute heterogeneous populations that differ their morphology, gene expression function. Although glial fibrillary acidic protein (GFAP) is cardinal cytological marker of CNS astrocytes, GFAP-negative astrocytes can easily be found adult CNS. also allocated spatially distinct regional domains during development. This heterogeneity suggests they help coordinate postnatal neural circuit formation...
Abstract Cross-talk between peripheral neurons and immune cells is important in pain sensation. We identified Snx25 as a pain-modulating gene transgenic mouse line with reduced sensitivity. Conditional deletion of monocytes macrophages, but not sensory neurons, mice ( cKO mice) responses both normal neuropathic conditions. Bone marrow transplantation using wild-type indicated that macrophages modulated Expression sorting nexin (SNX)25 dermal enhanced expression the neurotrophic factor NGF...
Upshaw-Schulman syndrome (USS) is a congenital thrombotic thrombocytopenic purpura (TTP) due to mutations in the gene that encodes for ADAMTS13 (ADAMTS13), but its clinical signs may be mild or absent during childhood. We have identified 37 patients with USS (24 females, 13 males) belonging 32 families. The nine women from six families who were diagnosed their first pregnancy are focus of this report. Six had episodes thrombocytopenia childhood misdiagnosed as idiopathic purpura....
Summary To clarify the pathogenic processes of thrombotic microangiopathies (TMAs) in patients with connective tissue disease (CTD), we analysed clinical characteristics and plasma ADAMTS13 levels 127 CTD-TMAs, including systemic lupus erythematosus (SLE), sclerosis, polymyositis/dermatomyositis, rheumatoid arthritis (RA), 64 acquired idiopathic thrombocytopenic purpura (ai-TTP). Plasma activity, antigen, inhibitors were determined by enzyme immunoassays. IgG type anti-ADAMTS13 antibodies...
Thrombotic thrombocytopenic purpura (TTP) is a type of thrombotic microangiopathy (TMA). Studies report that the majority TTP patients present with deficiency ADAMTS13 activity. In database TMA in Japan identified between 1998 and 2008, 186 first onset acquired idiopathic (ai) ADAMTS13-deficient (ADAMTS13 activity <5%) were diagnosed. The median age this group was 54 years, 54.8% female, 75.8% had renal involvement, 79.0% neurologic symptoms, 97.8% detectable inhibitors to Younger less...
A 68-year-old Japanese woman infected with influenza developed thrombotic thrombocytopenic purpura (TTP) 2 days after having a fever. Routine laboratory tests on admission suggested diagnosis of disseminated intravascular coagulation. However, ADAMTS13 assays showed an extremely low level plasma activity high titer anti-ADAMTS13 inhibitor (IgG). Despite high-dose methylprednisolone therapy daily exchange for 3 consecutive days, the patient died pulmonary congestion complicated by cardiac...
Oxidative stress contributes to myocardial ischemia-reperfusion injury, which causes cardiomyocyte death and precipitate life-threatening heart failure. Propofol has been proposed protect cells or tissues against oxidative stress. However, the mechanisms underlying its beneficial effects are not fully elucidated. In present study, we employed an in vitro injury model, rat cardiac H9c2 were treated with H2O2, investigated roles of propofol treatment reduced H2O2-induced apoptotic cell death....
Changes in astrocyte morphology are primarily attributed to the fine processes where intimate connections with neurons form tripartite synapse and participate neurotransmission. Recent evidence has shown that neurotransmission induces dynamic synaptic remodeling, suggesting astrocytic may adapt their morphologies activity environment. To illustrate such a neuron-glia relationship morophological detail, we employed double transgenic Olig2CreER/WT; ROSA26-GAP43-EGFP mice, which Olig2-lineage...
Plasma exchange (PE) is the first-line treatment for primary acquired thrombotic thrombocytopenic purpura (aTTP) with severe deficiency of ADAMTS13 activity (ADAMTS13:AC). Some patients are poor responders to PE, raising concern over multiple pathogenetic pathways.Based on 52 aTTP in our national cohort study, we monitored plasma levels ADAMTS13, clinical and laboratory findings, outcomes. In a representative responder examined an inhibitor (ADAMTS13:INH) complex milieu, by means large-pore...
Summary Congenital thrombotic thrombocytopenic purpura (cTTP), known as Upshaw–Schulman syndrome, is an ultrarare disorder caused by ADAMTS13 gene mutations; however, its long‐term outcomes have not been widely studied. A questionnaire survey was administered to physicians of patients in the Japanese cTTP registry characterise these outcomes. We analysed 55 remission, with 41 cases receiving prophylactic fresh frozen plasma (FFP; median dosage: 13·2 ml/kg per month) and 14 on‐demand FFP....
Circulating endotoxins including lipopolysaccharides (LPS) cause brain responses such as fever and decrease of food water intake, while pre-injection attenuates these responses. This phenomenon is called endotoxin tolerance, but the mechanisms underlying it remain unclear. The subfornical organ (SFO) rapidly produces proinflammatory cytokines interleukin-1β (IL-1β) in response to peripherally injected LPS, repeated LPS injection IL-1β production SFO, indicating that SFO involved tolerance....
Journal Article Proteolytic fragmentation and sugar chains of plasma ADAMTS13 purified by a conformation-dependent monoclonal antibody Get access Hisahide Hiura, Hiura 1Department Blood Transfusion Medicine, Nara Medical University, Kashihara, 634-8522; 2Department Biology, Faculty Technology, Fujita Health University School Sciences, Toyoake, Aichi 470-1192; 3Radioisotope Research Center, 634-8521, Japan Search for other works this author on: Oxford Academic PubMed Google Scholar Taei...
We describe an 18-year-old woman infected with H1N1 influenza followed by thrombotic microangiopathy. During the acute phase, her plasma levels of von Willebrand factor (VWF) were remarkably elevated, whereas those ADAMTS13 reduced without its inhibitors, generating a markedly high ratio VWF to in circulation. A retrospective analysis established following hypothesis: influenza-mediated cytokine storm induced enhanced release unusually large multimers (UL-VWFM) from vascular endothelial...
Background Thrombotic thrombocytopenic purpura ( TTP ) is characterized by deficient ADAMTS 13 activity. Treatment involves plasma exchange PE ). Both fresh‐frozen FFP and cryosupernatant CSP are used, but it remains to be determined which more effective. Study Design Methods To analyze the interaction between von Willebrand factor VWF 13, we used large‐pore isoelectric focusing IEF analysis followed detection with anti‐ ADAMT S13 monoclonal antibody. , cryoprecipitate CP ), purified were...
Abstract Influenza infections often cause pneumonia, but there is limited information on thrombotic microangiopathy ( TMA ) in these circumstances. We report the case of an 11‐year‐old boy who developed during acute phase H1N1 influenza. Plasma von Willebrand factor VWF was elevated, whereas a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13 ADAMTS 13) activity mildly reduced absence 13‐neutralizing autoantibody, resulting low ratio to . The patient treated...