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AIP56, an AB toxin secreted by Photobacterium damselae subsp. piscicida, has tr...

Freitas, IL; Macedo, MF; Oliveira, L; Oliveira, P; do Vale, A; Dos Santos, NMS

"Introduction: The AB-type toxin AIP56 is a key virulence factor of Photobacterium damselae subsp. piscicida (Phdp), inducing apoptosis in fish immune cells. The discovery of AIP56-like and AIP56-related toxins in diverse organisms, including human-associated Vibrio strains, highlights the evolutionary conservation of this toxin family, suggesting that AIP56 and its homologs may share conserved receptors across...


Acid sphingomyelinase deficiency: A clinical and immunological perspective

Pinto, C; Sousa, D; Ghilas, V; Dardis, A; Scarpa, M; Macedo, MF

Acid sphingomyelinase deficiency (ASMD) is a lysosomal storage disease caused by deficient activity of acid sphingomyelinase (ASM) enzyme, leading to the accumulation of varying degrees of sphingomyelin. Lipid storage leads to foam cell infiltration in tissues, and clinical features including hepatosplenomegaly, pulmonary insufficiency and in some cases central nervous system involvement. ASM enzyme replacement...


Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients

Pereira, CS; Perez-Cabezas, B; Ribeiro, H; Maia, M; Cardoso, M; Dias, AF; Azevedo, O; Ferreira, M; Garcia, P; Rodrigues, E; Castro-Chaves, P; Martins, E

The lysosome has a key role in the presentation of lipid antigens by CD1 molecules. While defects in lipid antigen presentation and in invariant Natural Killer T (iNKT) cell response were detected in several mouse models of lysosomal storage diseases (LSD), the impact of lysosomal engorgement in human lipid antigen presentation is poorly characterized. Here, we analyzed the capacity of monocyte-derived dendriti...


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