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Pregled bibliografske jedinice broj: 1220974

Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading


(de Matteis, Maria Antonietta ; Bender, Markus ; Swinkels, Maurice ; Versluis, Jurjen ; Jansen, A. J. Gerard) Bura, Ana; de Matteis, Maria Antonietta; Bender, Markus; Swinkels, Maurice; Versluis, Jurjen; Jansen, A. J. Gerard; Jurak Begonja, Antonija
Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading // British Journal of Haematology, (2022), 00; 1-13 doi:10.1111/bjh.18478 (međunarodna recenzija, članak, znanstveni)


CROSBI ID: 1220974 Za ispravke kontaktirajte CROSBI podršku putem web obrasca

Naslov
Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading

Autori
Bura, Ana ; de Matteis, Maria Antonietta ; Bender, Markus ; Swinkels, Maurice ; Versluis, Jurjen ; Jansen, A. J. Gerard ; Jurak Begonja, Antonija

Kolaboracija
De Matteis, Maria Antonietta ; Bender, Markus ; Swinkels, Maurice ; Versluis, Jurjen ; Jansen, A. J. Gerard

Izvornik
British Journal of Haematology (0007-1048) (2022), 00; 1-13

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
actin, bleeding disorders, Lowe syndrome, microtubules, nodules, OCRL, platelet function

Sažetak
Lowe syndrome (LS) is a rare, X-linked disorder characterised by numerous symp-toms affecting the brain, the eyes, and the kidneys. It is caused by mutations in the oculocerebrorenal syndrome of Lowe (OCRL) protein, a 5-phosphatase localised in different cellular compartments that dephosphorylates phosphatidylinositol-4, 5- bisphosphate into phosphatidylinositol-4- monophosphate. Some patients with LS also have bleeding disorders, with normal to low platelet (PLT) count and impaired PLT function. However, the mechanism of PLT dysfunction in patients with LS is not completely understood. The main function of PLTs is to activate upon vessel wall injury and stop the bleeding by clot formation. PLT activation is accompanied by a shape change that is a result of massive cytoskeletal rearrangements. Here, we show that OCRL-inhibited human PLTs do not fully spread, form mostly filopodia, and accumulate actin nodules. These nodules co-localise with ARP2/3 subunit p34, vinculin, and sorting nexin 9. Furthermore, OCRL-inhibited PLTs have a retained microtubular coil with high levels of acetylated tubulin. Also, myosin light chain phosphorylation is decreased upon OCRL inhibition, without impaired degranula-tion or integrin activation. Taken together, these results suggest that OCRL contributes to cytoskeletal rearrangements during PLT activation that could explain mild bleeding problems in patients with LS.

Izvorni jezik
Engleski

Znanstvena područja
Biologija, Temeljne medicinske znanosti, Biotehnologija u biomedicini (prirodno područje, biomedicina i zdravstvo, biotehničko područje)



POVEZANOST RADA


Projekti:
Ostalo-ASH-2019 - Nucleolar control of megakaryopoiesis (NucMeg) (Jurak Begonja, Antonija, Ostalo - American Society of Hematology (ASH) Global Research Award) ( POIROT)
HRZZ-DOK-2018-01-7426 - Projekt razvoja karijera mladih istraživača – izobrazba novih doktora znanosti (Jurak Begonja, Antonija, HRZZ ) ( POIROT)
HRZZ-UIP-2014-09-2400 - Uloga fosfoinozitida u nastanku trombocita (MkPI) (Jurak Begonja, Antonija, HRZZ - 2014-09) ( POIROT)
NadSve-Sveučilište u Rijeci-uniri-biomed-18-188-1343 - Identifikacija novih interakcijskih partnera Vps34 u megakariopoezi (Jurak Begonja, Antonija, NadSve - UNIRI projekti 2018) ( POIROT)

Ustanove:
Sveučilište u Rijeci - Odjel za biotehnologiju

Profili:

Avatar Url Ana Bura (autor)

Avatar Url Antonija Jurak Begonja (autor)

Poveznice na cjeloviti tekst rada:

doi pubmed.ncbi.nlm.nih.gov

Citiraj ovu publikaciju:

(de Matteis, Maria Antonietta ; Bender, Markus ; Swinkels, Maurice ; Versluis, Jurjen ; Jansen, A. J. Gerard) Bura, Ana; de Matteis, Maria Antonietta; Bender, Markus; Swinkels, Maurice; Versluis, Jurjen; Jansen, A. J. Gerard; Jurak Begonja, Antonija
Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading // British Journal of Haematology, (2022), 00; 1-13 doi:10.1111/bjh.18478 (međunarodna recenzija, članak, znanstveni)
(de Matteis, Maria Antonietta ; Bender, Markus ; Swinkels, Maurice ; Versluis, Jurjen ; Jansen, A. J. Gerard) (de Matteis, M., Bender, M., Swinkels, M., Versluis, J., Jansen, A. J. Gerard) Bura, Ana, de Matteis, M., Bender, M., Swinkels, M., Versluis, J., Jansen, A. & Jurak Begonja, A. (2022) Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading. British Journal of Haematology, (00), 1-13 doi:10.1111/bjh.18478.
@article{article, author = {Bura, Ana and de Matteis, Maria Antonietta and Bender, Markus and Swinkels, Maurice and Versluis, Jurjen and Jansen, A. J. Gerard and Jurak Begonja, Antonija}, year = {2022}, pages = {1-13}, DOI = {10.1111/bjh.18478}, keywords = {actin, bleeding disorders, Lowe syndrome, microtubules, nodules, OCRL, platelet function}, journal = {British Journal of Haematology}, doi = {10.1111/bjh.18478}, number = {00}, issn = {0007-1048}, title = {Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading}, keyword = {actin, bleeding disorders, Lowe syndrome, microtubules, nodules, OCRL, platelet function} }
@article{article, author = {Bura, Ana and de Matteis, Maria Antonietta and Bender, Markus and Swinkels, Maurice and Versluis, Jurjen and Jansen, A. J. Gerard and Jurak Begonja, Antonija}, year = {2022}, pages = {1-13}, DOI = {10.1111/bjh.18478}, keywords = {actin, bleeding disorders, Lowe syndrome, microtubules, nodules, OCRL, platelet function}, journal = {British Journal of Haematology}, doi = {10.1111/bjh.18478}, number = {00}, issn = {0007-1048}, title = {Oculocerebrorenal syndrome of Lowe protein controls cytoskeletal reorganisation during human platelet spreading}, keyword = {actin, bleeding disorders, Lowe syndrome, microtubules, nodules, OCRL, platelet function} }

Časopis indeksira:


  • Current Contents Connect (CCC)
  • Web of Science Core Collection (WoSCC)
    • Science Citation Index Expanded (SCI-EXP)
    • SCI-EXP, SSCI i/ili A&HCI
  • Scopus
  • MEDLINE


Citati:





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