Shwachman-Diamond syndrome marrow cells show abnormally increased apoptosis mediated through the Fas pathway.

Blood
Yigal Dror, M H Freedman

Abstract

Shwachman-Diamond syndrome (SDS) is an inherited bone marrow disorder with varying cytopenias and a strong predilection to myelodysplastic syndrome (MDS) and acute myeloid leukemia. Previously, it was found that the percentage of CD34(+) cells in bone marrow and the in vitro colony formation from CD34(+) cells of patients with SDS were markedly reduced. For these reasons, and because apoptosis is central in the pathogenesis of bone marrow dysfunction in MDS, this study was initiated to delineate the role of apoptosis in the pathogenesis of the marrow failure. Eleven children with SDS were studied. Compared to normal controls, patients' marrow mononuclear cells plated in clonogenic cultures showed a significantly higher tendency to undergo apoptosis. The defect in SDS was found in patients with and without MDS. Patients showed a more prominent decrease in colony formation and increased apoptosis after preincubation with activating anti-Fas antibody. Fas expression on marrow cells from patients was significantly higher than from normal controls. The difference between patients and controls for Fas expression was also significant for the following cell fraction subpopulations: CD34(-)/CD38(-), CD34(-)/CD38(+), and CD34(+). In conc...Continue Reading

References

Sep 1, 1981·The Journal of Pediatrics·W G WoodsW Krivit
Sep 1, 1995·British Journal of Haematology·J P MaciejewskiN S Young
Aug 1, 1996·British Journal of Haematology·O P SmithP Milla
Nov 1, 1996·British Journal of Haematology·P LepelleyP Fenaux
Jul 1, 1997·British Journal of Urology·C GranataD S O'Briain
Aug 26, 1998·Archives of Disease in Childhood·H StewartH Kingston
Aug 26, 1998·Leukemia & Lymphoma·P LepelleyP Fenaux
Aug 28, 1998·Science·A Ashkenazi, V M Dixit

❮ Previous
Next ❯

Citations

Nov 15, 2008·Apoptosis : an International Journal on Programmed Cell Death·Ken-Ichiro WatanabeYigal Dror
Aug 9, 2003·Current Allergy and Asthma Reports·Steven M Stein, David C Dale
Oct 15, 2003·Blood Reviews·Philip James Ancliff
May 24, 2003·Experimental Hematology·Andrew A G AprikyanDavid C Dale
Sep 10, 2003·Experimental Hematology·Shashikant Kulkarni, Monica Bessler
Mar 26, 2003·Seminars in Cancer Biology·Klaus Michael DebatinSimone Fulda
Apr 21, 2004·The Journal of Molecular Diagnostics : JMD·Megan S Lim, Kojo S J Elenitoba-Johnson
Dec 24, 2008·Expert Reviews in Molecular Medicine·Yigal Dror
Jan 26, 2007·Nature Reviews. Cancer·Seth J CoreyAaron D Schimmer
Mar 29, 2002·British Journal of Haematology·Ian ThornleyMelvin H Freedman
Aug 16, 2002·British Journal of Haematology·Yigal Dror, Melvin H Freedman
Apr 17, 2002·Seminars in Hematology·Melvin H Freedman, Blanche P Alter
Apr 17, 2002·Seminars in Hematology·O P Smith
Jun 10, 2003·Journal of Pediatric Hematology/oncology·Ashok B RajJoseph H Hersh
Nov 9, 2007·Current Opinion in Allergy and Clinical Immunology·Alejandro A Schäffer, Christoph Klein
Mar 8, 2008·The Journal of Clinical Investigation·Karyn M AustinAkiko Shimamura
Mar 10, 2004·International Journal of Hematology·Tetsuo MitsuiKiyoshi Hayasaka
Jan 27, 2006·PLoS Medicine·Joseph A Church
Sep 13, 2001·British Journal of Haematology·Y DrorM H Freedman
Mar 24, 2010·Pediatric Clinics of North America·Parinda MehtaFranklin O Smith
Mar 31, 2009·Hematology/oncology Clinics of North America·Lauri BurroughsAkiko Shimamura
Mar 31, 2009·Hematology/oncology Clinics of North America·Grover C Bagby, Gabrielle Meyers
Dec 26, 2006·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Daniel GarwiczBengt Fadeel
Oct 4, 2005·British Journal of Haematology·Simone CesaroUNKNOWN EBMT Severe Aplastic Anaemia and Paediatric Diseases Working Party
Jul 12, 2005·Pediatric Blood & Cancer·Fidel MajeedYigal Dror
Nov 20, 2008·British Journal of Haematology·Azim Mohamedali, Ghulam J Mufti
May 10, 2006·British Journal of Haematology·Elaine W LeungYigal Dror
Jul 28, 2005·Pediatric Blood & Cancer·Yigal Dror
Apr 16, 2008·British Journal of Haematology·Karthik A Ganapathi, Akiko Shimamura
Nov 11, 2010·Clinical and Experimental Immunology·S KhanA Huissoon
Jul 11, 2006·Seminars in Hematology·Akiko Shimamura
Oct 30, 2004·Hematology/oncology Clinics of North America·Yigal Dror, Lillian Sung
Mar 14, 2007·Orvosi hetilap·Melinda Erdos, László Maródi

❮ Previous
Next ❯

Related Concepts

Related Feeds

AML: Role of LSD1 by CRISPR (Keystone)

Find the latest rersearrch on the ability of CRISPR-Cas9 mutagenesis to profile the interactions between lysine-specific histone demethylase 1 (LSD1) and chemical inhibitors in the context of acute myeloid leukemia (AML) here.

Apoptosis

Apoptosis is a specific process that leads to programmed cell death through the activation of an evolutionary conserved intracellular pathway leading to pathognomic cellular changes distinct from cellular necrosis

Acute Myeloid Leukemia

Acute myeloid leukemia (AML) is a clinically and genetically heterogeneous disease with approximately 20,000 cases per year in the United States. AML also accounts for 15-20% of all childhood acute leukemias, while it is responsible for more than half of the leukemic deaths in these patients. Here is the latest research on this disease.