Cited by 149Hemoglobinopathies and Related DisordersBlood properties and coagulationHemoglobin structure and function

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Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease

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Manci, E. A.·2006·Blood

DOI 10.1182/blood-2005-07-2839PMC PMC1895417

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Abstract

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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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
DOI: 10.1182/blood-2005-07-2839
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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Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease

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