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Blood, 15 February 2006, Vol. 107, No. 4, pp. 1651-1658. Prepublished online as a Blood First Edition Paper on September 15, 2005; DOI 10.1182/blood-2005-07-2839. This Article Full Text (PDF) All Versions of this Article: 2005-07-2839v1 107/4/1651    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Manci, E. A Articles by Coller, B. S Search for Related Content PubMed PubMed Citation Articles by Manci, E. A Articles by Coller, B. S Related Collections Related Letter in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 21, 2005 Accepted August 26, 2005 Pathology of "Berkeley" sickle cell mice: similarities and differences with human sickle cell disease Elizabeth A Manci*, Cheryl A Hillery, Carol A Bodian, Zheng G Zhang, Gerald A Lutty, and Barry S Coller Department of Pathology, Centralized Pathology Unit for Sickle Cell Disease, University of South Alabama, Mobile, AL, USA Medical College of Wisconsin and the Blood Center of Southeast Wisconsin, Milwaukee, WI, USA Department of Anesthesiology, Mount Sinai School of Medicine, New York, NY, USA Department of Neurology, Henry Ford Hospital, Detroit, MI, USA Wilmer Ophthalmological Institute, Johns Hopkins Hospital, Baltimore, MD, USA The Laboratory of Blood and Vascular Biology, The Rockefeller University, New York, NY, USA * Corresponding author; email: emanci{at}usouthal.edu. Because "Berkeley" sickle cell mice are used as an animal model for human sickle cell disease, we investigated the progression of the histopathology in these animals over 6 months and compared these findings to those published in humans with sickle cell disease. The murine study groups were comprised of wild-type mixed C57Bl/6-SV129 (control) mice and sickle cell (SS) mice (-/-, -/-, transgene +) of both sexes and between 1 and 6 months of age. SS mice were similar to humans with sickle cell disease in having erythrocytic sickling, vascular ectasia, intravascular hemolysis, exuberant hematopoiesis, cardiomegaly, glomerulosclerosis, visceral congestion, hemorrhages, multiorgan infarcts, pyknotic neurons, and progressive siderosis. Cerebral perfusion studies demonstrated increased blood brain barrier permeability in SS mice. SS mice differed from humans with sickle cell disease in having splenomegaly; splenic hematopoiesis; more severe hepatic infarcts; less severe pulmonary manifestations; no significant vascular intimal hyperplasia; and only a trend toward vascular medial hypertrophy. Early retinal degeneration caused by a homozygous mutation (rd1) independent from that causing sickle hemoglobin was an incidental finding in some Berkeley mice. While our study reinforces the fundamental strength of this model, the notable differences warrant careful consideration when drawing parallels to human sickle cell disease. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Letter in Blood Online: Pathology of "Berkeley" sickle-cell mice includes gallstones and priapism Lewis Hsu, Bhalchandra Diwan, Jerrold M. Ward, and Constance T. Noguchi Blood 2006 107: 3414-3415. [Full Text] [PDF] This article has been cited by other articles: D. R. Archer, J. K. Stiles, G. W. Newman, A. Quarshie, L. L. Hsu, P. Sayavongsa, J. Perry, E. M. Jackson, and J. M. Hibbert C-Reactive Protein and Interleukin-6 Are Decreased in Transgenic Sickle Cell Mice Fed a High Protein Diet J. Nutr., June 1, 2008; 138(6): 1148 - 1152. [Abstract] [Full Text] [PDF] L. L. Hsu, H. C. Champion, S. A. Campbell-Lee, T. J. Bivalacqua, E. A. Manci, B. A. Diwan, D. M. Schimel, A. E. Cochard, X. Wang, A. N. Schechter, et al. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability Blood, April 1, 2007; 109(7): 3088 - 3098. [Abstract] [Full Text] [PDF] L. Hsu, B. Diwan, J. M. Ward, and C. T. Noguchi Pathology of "Berkeley" sickle-cell mice includes gallstones and priapism Blood, April 15, 2006; 107(8): 3414 - 3415. [Full Text] [PDF]

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