PMID: 11605164Oct 18, 2001Paper

Pathophysiology of sickle cell disease: role of cellular and genetic modifiers

Seminars in Hematology
M H Steinberg, G P Rodgers

Abstract

Sickle hemoglobin (HbS), caused by a point mutation in the beta-globin gene of hemoglobin, polymerizes when deoxygenated. The pathophysiology of sickle cell disease results from cellular defects caused directly by the hemoglobin mutation interacting with the environment and many other gene products--a few known, but most yet unidentified--a typical example of epistasis. How normal tissue perfusion is interrupted is complex and why the phenotype of sickle cell disease differs from patient to patient is poorly understood. We review the "classic" aspects of the pathophysiology of sickle cell disease and focus on known and potential modulators of the phenotype of this disorder.

References

Aug 11, 1992·American Journal of Hematology·S O Sowemimo-CokerR B Francis
Apr 1, 1992·Baillière's Clinical Haematology·R L Nagel, A F Fleming
Apr 1, 1992·Human Genetics·C OnerC Altay
Apr 1, 1990·Physiological Reviews·J S Pober, R S Cotran
Jan 1, 1991·The Journal of Clinical Investigation·R M BookchinV L Lew
Jan 1, 1990·Advances in Protein Chemistry·W A Eaton, J Hofrichter
Oct 1, 1990·Transplantation·J S Pober, R S Cotran
Jan 29, 1987·The New England Journal of Medicine·B A MillerS H Orkin
Aug 1, 1989·American Journal of Hematology·S O Sowemimo-CokerR B Francis
Mar 13, 1987·Science·C T Esmon
Feb 1, 1988·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·G M Rodgers
Dec 1, 1982·The Journal of Clinical Investigation·R P HebbelM H Steinberg
Jan 1, 1995·Annual Review of Physiology·D N Granger, R J Korthuis
Jun 9, 1994·The New England Journal of Medicine·O S PlattP P Klug
Jan 1, 1996·Annual Review of Physiology·C L Shovlin, J Scott
Mar 1, 1996·Blood Reviews·D K KaulR L Nagel
Dec 1, 1996·Hematology/oncology Clinics of North America·S K Ballas, N Mohandas
Dec 1, 1996·Hematology/oncology Clinics of North America·R M Bookchin, V L Lew
Sep 11, 1997·The New England Journal of Medicine·H F Bunn
Nov 27, 1997·The New England Journal of Medicine·A SoloveyR P Hebbel
Dec 5, 1998·American Journal of Hematology·S A Zimmerman, R E Ware
Apr 17, 1999·Molecular and Cellular Biology·H AsanoG Stamatoyannopoulos
Aug 31, 1999·Journal of Hypertension·P M Vanhoutte
Sep 25, 1999·Journal of Leukocyte Biology·L R LardA J Duits
Dec 23, 1999·Current Opinion in Hematology·P E NewburgerS M Weissman
May 29, 2000·American Journal of Human Genetics·J K PritchardP Donnelly
Jun 29, 2000·Baillière's Clinical Haematology·M H Steinberg

❮ Previous
Next ❯

Citations

Mar 1, 2008·Indian Journal of Hematology & Blood Transfusion : an Official Journal of Indian Society of Hematology and Blood Transfusion·Rakhee KarJasmina Ahluwalia
May 7, 2005·Prostaglandins, Leukotrienes, and Essential Fatty Acids·Hongmei RenMichael Crawford
Jun 3, 2004·Nature Reviews. Immunology·Seung-Yong Seong, Polly Matzinger
Aug 16, 2002·British Journal of Haematology·Iheanyi OkpalaBetty Cheung
Dec 20, 2011·Child Neuropsychology : a Journal on Normal and Abnormal Development in Childhood and Adolescence·N RuffieuxR Sztajzel
Nov 9, 2005·Hematology·Andreia Averci CanalliNicola Conran
Apr 25, 2002·Journal of Pediatric Hematology/oncology·Charles T Quinn, George R Buchanan
Dec 23, 2004·European Journal of Haematology·Chukwuma EzehIheanyi Okpala
Aug 17, 2005·Medical Principles and Practice : International Journal of the Kuwait University, Health Science Centre·Abdullah Kutlar
Nov 7, 2007·Laboratory Hematology : Official Publication of the International Society for Laboratory Hematology·Robert BagdasaryanDongsheng Xu
Mar 19, 2014·Trends in Genetics : TIG·Matthew R RobinsonPeter M Visscher
Jun 8, 2004·British Journal of Haematology·Jan SimakJaroslav G Vostal
Sep 27, 2015·Revista brasileira de hematologia e hemoterapia·Bruna Spinella Pierrot-GalloMaria Stella Figueiredo
Feb 11, 2009·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·Anna L TylerJason H Moore
Sep 2, 2010·American Journal of Hematology·Xandra W van den TweelKarin Fijnvandraat
Apr 23, 2010·Journal of the American Academy of Nurse Practitioners·Glenda M NinkovichPatricia T Alpert
Jan 8, 2014·Journal of Clinical Laboratory Analysis·Sanjay Kumar PandeyRenu Saxena
Jan 9, 2012·Journal of Biomedical Informatics·Daniel R MasysHoward P Levy
Jul 21, 2015·Revista brasileira de hematologia e hemoterapia·Claudia R Lustosa SouzaMaria Stella Figueiredo
Jun 5, 2015·Revista brasileira de hematologia e hemoterapia·Juliane Almeida MoreiraRomélia Pinheiro Gonçalves
Jan 18, 2015·Blood Cells, Molecules & Diseases·Perla VicariMaria Stella Figueiredo
Oct 11, 2005·Hematology/oncology Clinics of North America·Iheanyi E Okpala
Dec 14, 2016·Pediatric Blood & Cancer·Camilo VieiraMarilda Souza Goncalves
Dec 2, 2005·Current Opinion in Hematology·Iheanyi Okpala
Dec 10, 2008·Journal of Obstetrics and Gynaecology : the Journal of the Institute of Obstetrics and Gynaecology·I SarrisE Oteng-Ntim
Aug 22, 2015·The International Journal of Lower Extremity Wounds·Afsaneh Alavi, Robert S Kirsner
Jul 18, 2020·Nigerian Medical Journal : Journal of the Nigeria Medical Association·Ngozi Immaculata UgwuEjike Augustine Okoye
Oct 20, 2004·Advances in Skin & Wound Care·Jennifer T Trent, Robert S Kirsner
Aug 4, 2006·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·Adeboye H AdewoyeMartin H Steinberg
Jun 4, 2011·APMIS : Acta Pathologica, Microbiologica, Et Immunologica Scandinavica·Iheanyi OkpalaSagrario Herrada
Jul 25, 2006·Expert Opinion on Investigational Drugs·Iheanyi Okpala
Oct 24, 2002·Blood·Neil M MatsuiStephen H Embury

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.