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Sökning: onr:"swepub:oai:lup.lub.lu.se:724fa8b1-a356-4b36-8a86-8ee640a45ce4" > Calmodulin inhibito...

Calmodulin inhibitors improve erythropoiesis in Diamond-Blackfan anemia

Taylor, Alison M. (författare)
Boston Children's Hospital,Harvard University
Macari, Elizabeth R. (författare)
Harvard University,Boston Children's Hospital
Chan, Iris T. (författare)
Harvard University,Boston Children's Hospital
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Blair, Megan C. (författare)
Boston Children's Hospital,Harvard University
Doulatov, Sergei (författare)
Boston Children's Hospital,Harvard University
Vo, Linda T. (författare)
Harvard University,Boston Children's Hospital
Raiser, David M. (författare)
Boston Children's Hospital,Harvard University,Brigham and Women's Hospital / Harvard Medical School
Siva, Kavitha (författare)
Lund University,Lunds universitet,Stamcellscentrum (SCC),Avdelningen för stamcellsforskning,Institutionen för laboratoriemedicin,Medicinska fakulteten,Stamceller till röda blodkroppar,Forskargrupper vid Lunds universitet,Stem Cell Center,Division of stem cell research,Department of Laboratory Medicine,Faculty of Medicine,Stem Cells to Red Blood Cells,Lund University Research Groups
Basak, Anindita (författare)
Broad Institute,Boston Children's Hospital,Harvard University
Pirouz, Mehdi (författare)
Boston Children's Hospital,Harvard University
Shah, Arish N. (författare)
Massachusetts Institute of Technology
McGrath, Katherine (författare)
Harvard University,Boston Children's Hospital
Humphries, Jessica M. (författare)
Harvard University,Boston Children's Hospital
Stillman, Emma (författare)
Harvard University,Boston Children's Hospital
Alter, Blanche P. (författare)
National Cancer Institute, USA
Calo, Eliezer (författare)
Massachusetts Institute of Technology
Gregory, Richard I. (författare)
Harvard University,Boston Children's Hospital
Sankaran, Vijay G. (författare)
Broad Institute,Harvard University,Boston Children's Hospital
Flygare, Johan (författare)
Lund University,Lunds universitet,Stamcellscentrum (SCC),Avdelningen för stamcellsforskning,Institutionen för laboratoriemedicin,Medicinska fakulteten,Stamceller till röda blodkroppar,Forskargrupper vid Lunds universitet,LUCC: Lunds universitets cancercentrum,Övriga starka forskningsmiljöer,Stem Cell Center,Division of stem cell research,Department of Laboratory Medicine,Faculty of Medicine,Stem Cells to Red Blood Cells,Lund University Research Groups,LUCC: Lund University Cancer Centre,Other Strong Research Environments
Ebert, Benjamin L. (författare)
Boston Children's Hospital,Harvard University,Broad Institute
Zhou, Yi (författare)
Harvard University,Boston Children's Hospital
Daley, George Q. (författare)
Harvard University,Boston Children's Hospital
Zon, Leonard I. (författare)
Boston Children's Hospital,Harvard University,Howard Hughes Medical Institute
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 (creator_code:org_t)
American Association for the Advancement of Science (AAAS), 2020
2020
Engelska.
Ingår i: Science Translational Medicine. - : American Association for the Advancement of Science (AAAS). - 1946-6242 .- 1946-6234. ; 12:566
Relaterad länk:
http://dx.doi.org/10...
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https://www.ncbi.nlm...
https://lup.lub.lu.s...
https://doi.org/10.1...
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  • Tidskriftsartikel (refereegranskat)
Abstract Ämnesord
Stäng  
  • Diamond-Blackfan anemia (DBA) is a rare hematopoietic disease characterized by a block in red cell differentiation. Most DBA cases are caused by mutations in ribosomal proteins and characterized by higher than normal activity of the tumor suppressor p53. Higher p53 activity is thought to contribute to DBA phenotypes by inducing apoptosis during red blood cell differentiation. Currently, there are few therapies available for patients with DBA. We performed a chemical screen using zebrafish ribosomal small subunit protein 29 (rps29) mutant embryos that have a p53-dependent anemia and identified calmodulin inhibitors that rescued the phenotype. Our studies demonstrated that calmodulin inhibitors attenuated p53 protein amount and activity. Treatment with calmodulin inhibitors led to decreased p53 translation and accumulation but does not affect p53 stability. A U.S. Food and Drug Administration-approved calmodulin inhibitor, trifluoperazine, rescued hematopoietic phenotypes of DBA models in vivo in zebrafish and mouse models. In addition, trifluoperazine rescued these phenotypes in human CD34+ hematopoietic stem and progenitor cells. Erythroid differentiation was also improved in CD34+ cells isolated from a patient with DBA. This work uncovers a potential avenue of therapeutic development for patients with DBA.

Ämnesord

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Hematologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Hematology (hsv//eng)

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