ArticleThe American journal of pathology2012
Splenic morphological changes are accompanied by altered baseline immunity in a mouse model of sickle-cell disease.
Article in The American journal of pathology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 38 citations in OpenAlex.
- FermentedPharmaceutical biology · 2026Article
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- Immunomodulatory Effects ofPharmaceuticals (Basel, Switzerland) · 2025Article
- Preclinical efficacy of a modified gamma-globin lentivirus gene therapy in Berkeley sickle cell anemia mice and human xenograft models.Molecular therapy. Methods & clinical development · 2025Article
- Reduced GATA1 levels are associated with ineffective erythropoiesis in sickle cell anemia.Haematologica · 2025Article
- Article
- ΔThe Journal of pharmacology and experimental therapeutics · 2024Article
- Mouse models of sickle cell disease: Imperfect and yet very informative.Blood cells, molecules & diseases · 2024Review
- Limitations of mouse models for sickle cell disease conferred by their human globin transgene configurations.Disease models & mechanisms · 2022Article
- Resveratrol Protects against Restraint Stress Effects on Stomach and Spleen in Adult Male Mice.Animals : an open access journal from MDPI · 2019Article
- Robust adaptive immune response againstBlood advances · 2018Article
- Histological Disorganization of Spleen Compartments and Severe Visceral Leishmaniasis.Frontiers in cellular and infection microbiology · 2018Review
- Characterization of a mouse model of sickle cell trait: parallels to human trait and a novel finding of cutaneous sensitization.British journal of haematology · 2017Article
- Article
- Turf wars: exploring splenomegaly in sickle cell disease in malaria-endemic regions.British journal of haematology · 2017Review
- Adrenergic-mediated loss of splenic marginal zone B cells contributes to infection susceptibility after stroke.Nature communications · 2017Article
- Article
- The sickle cell mouse lung: proinflammatory and primed for allergic inflammation.Translational research : the journal of laboratory and clinical medicine · 2015Article
- Immunologic characterization suggests reduced alloimmunization in a murine model of thalassemia intermedia.Transfusion · 2014Article
- Transgenic sickle cell disease mice have high mortality and dysregulated immune responses after vaccination.Pediatric research · 2013Article
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Authors and funding
10 authors at 1 institution in 1 country.
Funding
Abstract
Although functional asplenia from infarctions may be a major contributor to increased infectious mortality in sickle-cell disease (SCD), this relationship has not been fully defined. We used the transgenic Berkeley SCD mouse to define blood and splenic immunophenotypic differences in this model compared with C57BL/6 and hemizygous controls. In the serum of SCD mice, we found increased IgG2a and suppressed IgM, IgG2b, and IgA levels. Serum IL-6 levels in SCD mice were elevated, whereas IL-1α, CXCL10, and CCL5 levels were decreased. The blood of SCD mice had higher white blood cell counts, with an increased percentage of lymphocytes and decreases in other leukocytes. Immunophenotyping of lymphocytes revealed higher percentages of CD8(+) and T-regulatory cells and lower percentages of B cells. SCD mouse spleens exhibited histological disorganization, with reduction of defined lymphoid follicles and expansion of red pulp, a greater than fourfold increase in splenic mononuclear cells, marked expansion of the nucleated red blood cell fraction, and B-cell and CD8(+) T-cell lymphopenia. Within the splenic B-cell population, there was a significant decrease in B-1a B cells, with a corresponding decrease in IgA secreting plasma cells in the gut. Confocal microscopy of spleens demonstrated complete disruption of the normal lymphofollicular structure in the white pulp of SCD mice without distinct B, T, and marginal zones. Our findings suggest that altered SCD splenic morphological characteristics result in an impaired systemic immune response.
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