Publications by authors named "Bryan C Y Au"
Background: Dendritic cells (DCs) are promising mediators of anti-tumor immune responses due to their potent antigen-presentation capacity. Unfortunately, cancer cells can often disarm differentiated DCs by rendering them incapable of maturation or by promoting their apoptosis. DC vaccine regimens attempt to generate functional DCs and preload them with Tumor-Associated Antigens (TAAs) to target various malignancies.
View Article and Find Full Text PDFFarber disease (FD) is a severe inherited disorder of lipid metabolism characterized by deficient lysosomal acid ceramidase (ACDase) activity, resulting in ceramide accumulation. Ceramide and metabolites have roles in cell apoptosis and proliferation. We introduced a single-nucleotide mutation identified in human FD patients into the murine Asah1 gene to generate the first model of systemic ACDase deficiency.
View Article and Find Full Text PDFArticle Synopsis
- Fabry disease is a genetic disorder marked by a lack of the enzyme α-galactosidase A, leading to harmful buildup of globotriaosylceramide (Gb(3)) in various organs.
- Researchers created a special mouse model that minimizes immune response and has low natural enzyme activity to study potential human therapies for this condition.
- In experiments, mice receiving a human gene therapy showed higher enzyme activity and notably reduced Gb(3) levels in critical organs like the heart and kidneys, compared to those receiving a control treatment.