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Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease
Open accessAbstract
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 composed of wild-type mixed C57Bl/6-SV129 (control) mice and sickle cell (SS) mice (alpha-/-, beta-/-, 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.
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“Young sickle mice do not commonly develop large-vessel vasculopathy and stroke (153, 154)”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [153]
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Cited by
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Pathophysiology of Sickle Cell Disease
Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012418-012838 · Cited by 813
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Sickle cell disease: old discoveries, new concepts, and future promise
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Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice
Belcher, J. D. (2006) · Journal of Clinical Investigation · doi:10.1172/jci26857 · Cited by 267
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Metadata
- Authors
- Manci, E. A.
- Affiliations
- Johns Hopkins University (US)
- Corresponding author
- Manci, E. A.
- ORCID
- Manci, E. A.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2006-02-15
- Volume
- 107
- Issue
- 4
- Pages
- 1651-1658
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- PMC
- PMC1895417
- OpenAlex
- W2066774936
- Cited by
- 149
- References
- 48
- Citations by year
- 2026: 5 · 2025: 7 · 2024: 2 · 2023: 11 · 2022: 8 · 2021: 10 · 2020: 9 · 2019: 10 · 2018: 12 · 2017: 10 · 2016: 10 · 2015: 9 · 2014: 7 · 2013: 5 · 2012: 5
- FWCI
- 1.93
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Stony Brook University
- National Institutes of Health (n01hb007086-007)
- National Heart, Lung, and Blood Institute (P01HL044612, HL70981, HL 19278, N0-1-HB07086, R01HL070981, HL-44612, HL45922, R01HL019278, R01HL045922)
- National Institute of Neurological Disorders and Stroke (NS 43324, R01NS043324)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Hematology › Blood properties and coagulation
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Hemoglobin structure and function
- Keywords
- sickle cell disease, animal models, histopathology, angiodysplasia, intravascular hemolysis, cardiomegaly, glomerulosclerosis, splenomegaly, retinal degeneration, siderosis
- MeSH terms
- Anemia, Sickle Cell, Animals, Body Weight, Humans, Mutation, Organ Size, Reference Values, Species Specificity, Mice
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