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  1. Article ; Online: Platelet production by megakaryocytes: protoplatelet theory justifies cytoplasmic fragmentation model.

    Kosaki, Goro

    International journal of hematology

    2008  Volume 88, Issue 3, Page(s) 255–267

    Abstract: The maturation of megakaryocytes (MKs) leading to platelet production is carefully reviewed. For instance, when MK with ploidy 16N enters the maturation stage, eight centrosomes are clustered in the cell center surrounded by 16N nucleus. Each bundle of ... ...

    Abstract The maturation of megakaryocytes (MKs) leading to platelet production is carefully reviewed. For instance, when MK with ploidy 16N enters the maturation stage, eight centrosomes are clustered in the cell center surrounded by 16N nucleus. Each bundle of microtubules (MTs) emanated from the respective centrosome supports and organize eight equally volumed cytoplasmic compartments which together compose one single 16N MK. Then, the following three processes take place in parallel until the complete maturation of MKs Virchow's Arch. 1906;186:55-63. Two centrioles, composing centrosome, are separated and each one with pericentriolar material migrates to just beneath the plasma membrane through the MT bundle [corresponding to a half of the interphase array, originated from one centrosome, supporting one "putative cytoplasmic compartment "(PCC)] (Blood. 2005;106:4066-75). Platelet specific granules and other cellular components, newly formed in the central field of the cell, are transported along the MTs and many platelet territories, future platelets, are elaborated as a tandem array from the center to periphery in each PCC [3]. All the important membranes including plasma membrane and platelet demarcation membrane (DM) are synthesized de novo and those are transported as membrane vesicles (MVs) from Golgi body along the MTs. MVs arranged on the boundary surface between neighboring PCCs undergo fusion and fission to yield a paired membrane. Further connection with the external milieu results in the completion of DM system. The PCC covered by a sheet of DM is designated as protoplatelet. Excessive production of the MVs, most probably intervenes between the respective protoplatelets. Eventually, the matured MK ruptures as a whole, resulting in release of platelets from protoplatelets and many of MVs, though the mechanism is not fully elucidated yet.
    MeSH term(s) Animals ; Blood Platelets/cytology ; Blood Platelets/physiology ; Humans ; Megakaryocytes/cytology ; Megakaryocytes/physiology ; Models, Biological ; Thrombopoiesis/physiology
    Language English
    Publishing date 2008-08-28
    Publishing country Japan
    Document type Journal Article ; Review
    ZDB-ID 1076875-0
    ISSN 1865-3774 ; 0917-1258 ; 0925-5710
    ISSN (online) 1865-3774
    ISSN 0917-1258 ; 0925-5710
    DOI 10.1007/s12185-008-0147-7
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 269: Show issues Location:
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  2. Article ; Online: Interstitial Pneumonia Secondary to Hermansky-Pudlak Syndrome Type 4 Treated with Different Antifibrotic Agents.

    Itano, Junko / Tanimoto, Yasushi / Kimura, Goro / Hamada, Noboru / Tanaka, Hisaaki / Ninomiya, Shinsuke / Kosaki, Kenjiro / Miyahara, Nobuaki / Maeda, Yoshinobu / Kiura, Katsuyuki

    Internal medicine (Tokyo, Japan)

    2021  Volume 60, Issue 5, Page(s) 783–788

    Abstract: Hermansky-Pudlak syndrome (HPS) is an autosomal recessive hereditary disease that may be complicated by progressive and potentially fatal interstitial pneumonia. We herein report a 64-year-old woman with interstitial pneumonia associated with HPS type 4 ... ...

    Abstract Hermansky-Pudlak syndrome (HPS) is an autosomal recessive hereditary disease that may be complicated by progressive and potentially fatal interstitial pneumonia. We herein report a 64-year-old woman with interstitial pneumonia associated with HPS type 4 whom we treated with nintedanib after pirfenidone proved ineffective. To our knowledge, there have been no previous reports of nintedanib being used to treat a patient with HPS type 4. There is a need for clinical trials of antifibrotic agents, including nintedanib, pirfenidone, and new therapeutic agents with different mechanisms of action in these patients.
    MeSH term(s) Female ; Hermanski-Pudlak Syndrome ; Humans ; Lung Diseases, Interstitial/drug therapy ; Lung Diseases, Interstitial/etiology ; Middle Aged ; Pulmonary Fibrosis/drug therapy ; Pulmonary Fibrosis/etiology
    Language English
    Publishing date 2021-01-20
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 32371-8
    ISSN 1349-7235 ; 0021-5120 ; 0918-2918
    ISSN (online) 1349-7235
    ISSN 0021-5120 ; 0918-2918
    DOI 10.2169/internalmedicine.5493-20
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 240: Show issues Location:
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  3. Article ; Online: Noonan syndrome-like phenotype in a patient with heterozygous ERF truncating variant.

    Yamada, Mamiko / Funato, Michinori / Kondo, Goro / Suzuki, Hisato / Uehara, Tomoko / Takenouchi, Toshiki / Sakamoto, Yoshiaki / Kosaki, Kenjiro

    Congenital anomalies

    2021  Volume 61, Issue 6, Page(s) 226–230

    Abstract: Craniosynostosis is caused by abnormalities of multiple signaling pathways, including excessive RAS signaling. Recently, a truncating variant in ETS2 repressor factor (ERF), a negative transcriptional regulator of the RAS pathway, was shown to be ... ...

    Abstract Craniosynostosis is caused by abnormalities of multiple signaling pathways, including excessive RAS signaling. Recently, a truncating variant in ETS2 repressor factor (ERF), a negative transcriptional regulator of the RAS pathway, was shown to be associated with craniosynostosis. Here, we report a 10-year-old male patient with a heterozygous nonsense mutation, p.Arg183*, in ERF who exhibited craniosynostosis with Noonan syndrome-like phenotypes. In consideration that loss-of-function variants in ERF would result in excessive RAS signaling and RASopathy phenotypes, we propose that ERF may represent a causative gene for Noonan syndrome. Since preceding studies on ERF mutations dealt with patients who were ascertained because of craniosynostosis, further studies are needed to evaluate whether patients with variants in ERF can present with Noonan syndrome-like features without craniosynostosis.
    MeSH term(s) Child ; Craniosynostoses/genetics ; Heterozygote ; Humans ; Male ; Mutation ; Noonan Syndrome/diagnosis ; Noonan Syndrome/genetics ; Phenotype ; Repressor Proteins
    Chemical Substances ERF protein, human ; Repressor Proteins
    Language English
    Publishing date 2021-07-11
    Publishing country Australia
    Document type Case Reports ; Journal Article
    ZDB-ID 228664-6
    ISSN 1741-4520 ; 0037-2285 ; 0914-3505
    ISSN (online) 1741-4520
    ISSN 0037-2285 ; 0914-3505
    DOI 10.1111/cga.12435
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 1272: Show issues Location:
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  4. Article: In vivo platelet production from mature megakaryocytes: does platelet release occur via proplatelets?

    Kosaki, Goro

    International journal of hematology

    2005  Volume 81, Issue 3, Page(s) 208–219

    Abstract: Although platelets are universally accepted to be born from megakaryocytes (MKs), the mechanism by which platelets are formed and released from MKs in vivo remains controversial. One theory, known as the proplatelet theory, postulates that platelets are ... ...

    Abstract Although platelets are universally accepted to be born from megakaryocytes (MKs), the mechanism by which platelets are formed and released from MKs in vivo remains controversial. One theory, known as the proplatelet theory, postulates that platelets are released from proplatelet processes protruding from MKs into sinusoids located in the bone marrow hematopoietic compartment. Proplatelet formation (PPF) has been observed in in vitro experiments involving detailed analyses of related molecular events. PPF has also been used as a marker of MK maturation. However, PPF is suggested to be a nonphysiological phenomenon. On the other hand, transmission electron microscopy (TEM) analyses have revealed platelet formation via explosive fragmentation of MK cytoplasm in bone marrow and lung capillaries prepared by immersion fixation. Moreover, TEM and scanning electron microscopy studies of liquid-cultured MKs kept in suspension show that platelet formation occurs without PPF. Rather, an explosive and global fragmentation of the MK cytoplasm composed of platelet territories has been reported as the mechanism of platelet formation. In addition, in vivo and ex vivo observations of platelet release from MKs with phase-contrast microscopy strongly support the explosive-fragmentation theory. With all observations taken into account, PPF may not be a prerequisite for platelet release from MKs under real-life conditions. In this review, a new "protoplatelet" concept is proposed to support the explosive-fragmentation theory. Additionally, the role of the lungs in platelet production is reviewed and discussed.
    MeSH term(s) Animals ; Blood Platelets/cytology ; Blood Platelets/physiology ; Bone Marrow/physiology ; Capillaries/physiology ; Humans ; Lung/blood supply ; Lung/physiology ; Megakaryocytes/cytology ; Megakaryocytes/physiology ; Thrombopoiesis/physiology
    Language English
    Publishing date 2005-04
    Publishing country Japan
    Document type Journal Article ; Review
    ZDB-ID 1076875-0
    ISSN 1865-3774 ; 0925-5710 ; 0917-1258
    ISSN (online) 1865-3774
    ISSN 0925-5710 ; 0917-1258
    DOI 10.1532/IJH97.04177
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 269: Show issues Location:
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  5. Article ; Online: Thrombocytogenesis by megakaryocyte; Interpretation by protoplatelet hypothesis.

    Kosaki, Goro / Kambayashi, Junichi

    Proceedings of the Japan Academy. Series B, Physical and biological sciences

    2011  Volume 87, Issue 5, Page(s) 254–273

    Abstract: Serial transmission electron microscopy of human megakaryocytes (MKs) revealed their polyploidization and gradual maturation through consecutive transition in characteristics of various organelles and others. At the beginning of differentiation, MK with ... ...

    Abstract Serial transmission electron microscopy of human megakaryocytes (MKs) revealed their polyploidization and gradual maturation through consecutive transition in characteristics of various organelles and others. At the beginning of differentiation, MK with ploidy 32N, e.g., has 16 centrosomes in the cell center surrounded by 32N nucleus. Each bundle of microtubules (MTs) emanated from the respective centrosome supports and organizes 16 equally volumed cytoplasmic compartments which together compose one single 32N MK. During the differentiation, single centriole separated from the centriole pair, i.e., centrosome, migrates to the most periphery of the cell through MT bundle, corresponding to a half of the interphase array originated from one centrosome, supporting one "putative cytoplasmic compartment" (PCC). Platelet demarcation membrane (DM) is constructed on the boundary surface between neighbouring PCCs. Matured PCC, composing of a tandem array of platelet territories covered by a sheet of DM is designated as protoplatelet. Eventually, the rupture of MK results in release of platelets from protoplatelets. (Communicated by Tadamitsu Kishimoto, M.J.A.).
    MeSH term(s) Blood Platelets/cytology ; Blood Platelets/ultrastructure ; Cell Differentiation ; Centrioles/metabolism ; Centrioles/ultrastructure ; Humans ; Megakaryocytes/cytology ; Megakaryocytes/ultrastructure ; Models, Biological ; Polyploidy
    Language English
    Publishing date 2011-05-11
    Publishing country Japan
    Document type Journal Article ; Review
    ZDB-ID 161781-3
    ISSN 1349-2896 ; 0386-2208
    ISSN (online) 1349-2896
    ISSN 0386-2208
    DOI 10.2183/pjab.87.254
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Bonn / Germany

    Z 4' 46/60 b: Show issues
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  6. Article ; Online: Two patients with Rubinstein-Taybi syndrome and severe pulmonary interstitial involvement.

    Kosaki, Rika / Kikuchi, Shintaro / Koinuma, Goro / Higuchi, Masataka / Torii, Chiharu / Kawasaki, Kazuteru / Kosaki, Kenjiro

    American journal of medical genetics. Part A

    2010  Volume 152A, Issue 7, Page(s) 1844–1846

    MeSH term(s) Fatal Outcome ; Female ; Humans ; Infant ; Infant, Newborn ; Lung Diseases, Interstitial/complications ; Lung Diseases, Interstitial/diagnostic imaging ; Male ; Pregnancy ; Radiography, Thoracic ; Rubinstein-Taybi Syndrome/complications ; Rubinstein-Taybi Syndrome/diagnostic imaging
    Language English
    Publishing date 2010-06-26
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1493479-6
    ISSN 1552-4833 ; 1552-4825
    ISSN (online) 1552-4833
    ISSN 1552-4825
    DOI 10.1002/ajmg.a.33382
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Book: Geka shindangaku

    Kōsaki, Gorō

    1988  

    Author's details henshū Kōsaki Gorō, Wada Tatsuo, Abe Osahiko
    MeSH term(s) Diagnostic Techniques, Surgical
    Language Japanese
    Size xviii, 636 p. :, ill.
    Edition Dai 1-han.
    Publisher Ishiyaku Shuppan
    Publishing place Tōkyō
    Document type Book
    Database Catalogue of the US National Library of Medicine (NLM)

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  8. Article: Upper airway obstruction in neonates and infants with CHARGE syndrome.

    Naito, Yoko / Higuchi, Masataka / Koinuma, Goro / Aramaki, Michihiko / Takahashi, Takao / Kosaki, Kenjiro

    American journal of medical genetics. Part A

    2007  Volume 143A, Issue 16, Page(s) 1815–1820

    Abstract: Upper airway obstruction can be life-threatening in neonates and infants with CHARGE syndrome, many of whom undergo intratracheal intubation early in life. Although some of these patients are successfully extubated, others require tracheotomy. Deciding ... ...

    Abstract Upper airway obstruction can be life-threatening in neonates and infants with CHARGE syndrome, many of whom undergo intratracheal intubation early in life. Although some of these patients are successfully extubated, others require tracheotomy. Deciding whether to complete tracheotomy is challenging since there are no clear criteria upon which to base this decision. We assessed 10 infants with CHARGE syndrome, 5 of whom required tracheotomy. Fiberoptic laryngoscopy showed that all of these patients shared certain features: anteroposterior flattening of the larynx; short vocal cords; anteriorly positioned, tall and hypertrophic arytenoids obscuring the glottis; uncoordinated movement of the vocal cords, epiglottis and arytenoids; salivary pooling. In addition, we observed only in those requiring tracheotomy an obstructive supraglottis that prevented visualization of the vocal cords throughout respiration. Salivary retention was much more severe in this group. These findings might be helpful for predicting the need for an early tracheotomy in situations where the vocal cords are not visible throughout the entire respiratory cycle. Given the high prevalence of malformed larynx and abnormal cranial nerve function, which are not alleviated by supraglottoplasty, we suggest that a thorough investigation of upper airway obstructive entities other than laryngomalacia be performed before embarking on supraglottoplasty in patients with CHARGE syndrome.
    MeSH term(s) Abnormalities, Multiple/pathology ; Airway Obstruction/diagnosis ; Airway Obstruction/pathology ; DNA Helicases/genetics ; DNA-Binding Proteins/genetics ; Epiglottis/physiopathology ; Humans ; Infant ; Infant, Newborn ; Laryngeal Diseases/pathology ; Laryngoscopy ; Larynx/abnormalities ; Larynx/pathology ; Mutation ; Syndrome ; Vocal Cords/physiopathology
    Chemical Substances DNA-Binding Proteins ; DNA Helicases (EC 3.6.4.-) ; CHD7 protein, human (EC 3.6.4.12)
    Language English
    Publishing date 2007-05-14
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2108614-X
    ISSN 1552-4825
    ISSN 1552-4825
    DOI 10.1002/ajmg.a.31851
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    Zs.A 1376/A: Show issues Location:
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  9. Book: Sui bunpisei toripushin inhibitā

    Kōsaki, Gorō

    kiso to rinshō

    1985  

    Author's details henshū Kōsaki Gorō, Ogawa Michio
    MeSH term(s) Trypsin Inhibitors
    Language Japanese
    Size 3, 310 p. :, ill.
    Edition Dai 1-han.
    Publisher Igaku Tosho Shuppan
    Publishing place Tōkyō
    Document type Book
    ISBN 9784871511186 ; 4871511189
    Database Catalogue of the US National Library of Medicine (NLM)

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  10. Book: DIC, shindan to chiryō

    Kōsaki, Gorō

    shōrei o chūshin to shite

    1984  

    Author's details Kōsaki Gorō, Maekawa Tadashi, Maki Masahiro hen
    MeSH term(s) Disseminated Intravascular Coagulation
    Language Japanese
    Size 320 p. :, ill.
    Publisher Gendai Iryōsha
    Publishing place Tōkyō
    Document type Book
    ISBN 9784905915126 ; 4905915124 ; 4905915120
    Database Catalogue of the US National Library of Medicine (NLM)

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