γD318Y fibrinogen shows no fibrin polymerization due to defective "A-a" and "B-b" interactions, whereas that of γK321E fibrinogen is nearly normal

ArticleThrombosis research. 182: 150-158. (2019)journal articl

Fibrin monomers derived from thrombogenic dysfibrinogenemia, Naples-type variant (BβAla68Thr), showed almost entirely normal polymerization

ArticleTHROMBOSIS RESEARCH.172:1-3(2018)journal articl

Heterozygous Variant Fibrinogen γA289V (Kanazawa III) Was Confirmed as Hypodysfibrinogenemia by Plasma and Recombinant Fibrinogens

Introduction: Congenital fibrinogen disorders are classified as afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, and hypodysfibrinogenemia. However, difficulties are associated with discriminating between dysfibrinogenemia, hypofibrinogenemia, and hypodysfibrinogenemia using routine analyses. We previously reported a heterozygous variant fibrinogen (γA289V; Kanazawa III) as hypodysfibrinogenemia; however, the same variant had previously been described as hypofibrinogenemia. To clarify the production of γA289V fibrinogen, we expressed recombinant γA289V (r-γA289V) fibrinogen and compared it with wild-type (WT) and adjacent recombinant variant fibrinogens. Methods: Target mutations were introduced into a fibrinogen γ-chain expression vector by site-directed mutagenesis, and the vector was then transfected into Chinese hamster ovary cells to produce recombinant fibrinogen. Fibrinogen was purified from the plasma of the proposita, and culture media and fibrinogen functions were analyzed using fibrin polymerization, plasmin protection, and FXIIIa-catalyzed fibrinogen cross-linking. Results: The fibrinogen concentration ratio of the culture media to cell lysates was markedly lower for r-γA289V fibrinogen than for WT. Because the secretion of recombinant γF290L (r-γF290L) fibrinogen was similar to WT, we compared r-γF290L fibrinogen functions with WT. The fibrin polymerization of Kanazawa III plasma (K-III) fibrinogen was significantly weaker than normal plasma fibrinogen. Moreover, K-III fibrinogen showed a markedly reduced “D:D” interaction. However, all functions of r-γF290L fibrinogen were similar to WT. An in silico analysis confirmed the above results. Conclusion: The present results demonstrated that γA289 is crucial for the γ-module structure, and the γA289V substitution markedly reduced fibrinogen secretion. Moreover, K-III fibrinogen showed markedly reduced fibrin polymerization and “D:D” interactions. γA289V fibrinogen was confirmed as hypodysfibrinogenemia.ArticleINTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY.42(2):190-197(2020)journal articl

Congenital fibrinogen disorder with a compound heterozygote possessing two novel FGB mutations, one qualitative and the other quantitative

ArticleThrombosis research. 196: 152-158. (2020)journal articl

Hydrogen bonds of DsrD protein revealed by neutron crystallography

Hydrogen bonds of DNA-binding protein DsrD have been determined by neutron diffraction. In terms of proton donors and acceptors, DsrD protein shows striking differences from other proteins

A Novel Amino Acid Substitution, Fibrinogen Bβp.Pro234Leu, Associated with Hypofibrinogenemia Causing Impairment of Fibrinogen Assembly and Secretion

ArticleInternational journal of molecular sciences. 21(24): 9422(2020)journal articl

Novel variant fibrinogen γp.C352R produced hypodysfibrinogenemia leading to a bleeding episode and failure of infertility treatment

ArticleInternational journal of hematology. 114(3): 325-333. (2021)journal articl

DME-Fired Water-Tube Boiler - A R&D Study

Increasing attention has been given to the development of low-NOx combustion technology for DME (Dimethyl Ether). The present paper describes the R&D study for water-tube boiler carried out in Kansai University and Hirakawa Guidam Co., Ltd. under the support of DME project from METI. The major problem in DME use is the difficulty in the application of premixed flame due to its low ignition temperature and rather high burning velocity. However, the previously developed tube-nested combustor, i.e. water-tubes installed in the empty furnace, becomes effective means together with the flue-gas recirculation to overcome such difficulty in achieving low-NOx combustion. This paper begins with a brief review of the R&D study of the tube-nested combustor specifically designed for city gas. Then the further development for DME-fired water-tube boiler is described

Bladder dysfunction and urinary tract infection caused by abdominal pseudocyst with a ventriculoperitoneal shunt: A case report with literature review

The lower urinary tract dysfunction of the neurogenic bladder often worsens owing to dysfunction of the bladder itself. We treated a patient with spina bifida who experienced bladder dysfunction that worsened owing to an abdominal pseudocyst that developed as a complication of a ventriculoperitoneal shunt. After removal of the pseudocyst, the urinary tract infection was controlled and bladder dysfunction was alleviated to a basal level