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- W4313386026 startingPage "122.7" @default.
- W4313386026 abstract "Abstract Propionic Acidemia (PA) is a recessive disorder resulting from mutations in the PCCA or PCCB genes encoding the two subunits of propionyl-CoA carboxylase. Patients with PA generate propionate and potentially toxic metabolites after consumption of certain amino acids and odd-chain fatty acids leading to systemic health complications. In addition, PA patients are exceptionally sensitive to infections due to a compromised immune system. We developed a hypomorph mouse model of PA (Pcca-/-(A138T) that recapitulates many phenotypes associated with the human disease. In this study, we tested A138T mouse immune function and their susceptibility to infections. A138T mice had an overall reduction in white blood cells, lymphocytes, monocytes, granulocytes, and immunoglobulins when compared to control mice. When wild type and A138T mice were infected i.v. with adenovirus 5 (Ad5) expressing GFP-Luciferase, we observed higher luciferase expression in the livers of the Pcca-/-(A138T) suggesting that the mutant mice were more susceptible to the virus due to defects in the reticuloendothelial system. The A138T mice also generated lower levels of anti-GFP antibodies than control mice 7 weeks after injection suggesting weaker adaptive immune responses. Overall, our results show that the Pcca-/-(A138T) mouse model recapitulates the immunodefiency seen in humans and can be used to further explore their susceptibility to infections as well as the benefits or dangers of vaccination to PA patients." @default.
- W4313386026 created "2023-01-06" @default.
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- W4313386026 date "2015-05-01" @default.
- W4313386026 modified "2023-09-25" @default.
- W4313386026 title "Immunodeficiency in a hypomorphic mouse model of propianic acidemia (HUM4P.300)" @default.
- W4313386026 doi "https://doi.org/10.4049/jimmunol.194.supp.122.7" @default.
- W4313386026 hasPublicationYear "2015" @default.
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