Scopus İndeksli Yayınlar Koleksiyonu / Scopus Indexed Publications Collection
Permanent URI for this collectionhttps://hdl.handle.net/11147/7148
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Article Yeast-Produced Human Recombinant Lysosomal Β-Hexosaminidase Efficiently Rescues Gm2 Ganglioside Accumulation in Tay–Sachs Disease(Multidisciplinary Digital Publishing Institute (MDPI), 2025) Inci, O.K.; Leal, A.F.; Ates, N.; Súarez, D.A.; Espejo-Mojica, A.J.; Alméciga-Diaz, C.J.; Seyrantepe, V.Background: Tay–Sachs disease (TSD) is an autosomal recessive lysosomal storage disorder characterized by the accumulation of GM2 ganglioside due to mutations in the HEXA gene, which encodes the α-subunit of β-Hexosaminidase A. This accumulation leads to significant neuropathological effects and premature death in affected individuals. No effective treatments exist, but enzyme replacement therapies are under investigation. In our previous work, we demonstrated the internalization and efficacy of human recombinant lysosomal β-hexosaminidase A (rhHex-A), produced in the methylotrophic yeast Pichia pastoris, in reducing lipids and lysosomal mass levels in fibroblasts and neural stem cells derived from patient-induced pluripotent stem cells (iPSCs). In this study, we further evaluated the potential of rhHex-A to prevent GM2 accumulation using fibroblast and neuroglia cells from a TSD patient alongside a relevant mouse model. Methods: Fibroblasts and neuroglial cell lines derived from a murine model and TSD patients were treated with 100 nM rhHexA for 72 h. After treatment, cells were stained by anti-GM2 (targeting GM2 ganglioside; KM966) and anti-LAMP1 (lysosomal-associated membrane protein 1) colocalization staining and incubated with 50 nM LysoTracker Red DND-99 to label lysosomes. In addition, GM2AP and HEXB expression were analyzed to assess whether rhHex-A treatment affected the levels of enzymes involved in GM2 ganglioside degradation. Results: Immunofluorescence staining for LysoTracker and colocalization studies of GM2 and Lamp1 indicated reduced lysosomal mass and GM2 levels. Notably, rhHex-A treatment also affected the expression of the HEXB gene, which is involved in GM2 ganglioside metabolism, highlighting a potential regulatory interaction within the metabolic pathway. Conclusions: Here, we report that rhHex-A produced in yeast can efficiently degrade GM2 ganglioside and rescue lysosomal accumulation in TSD cells. © 2025 by the authors.Article Citation - Scopus: 20Estrus Detection and Dairy Cow Identification With Cascade Deep Learning for Augmented Reality-Ready Livestock Farming(Multidisciplinary Digital Publishing Institute (MDPI), 2023) Arıkan, İ.; Ayav, T.; Seçkin, A.Ç.; Soygazi, F.Accurate prediction of the estrus period is crucial for optimizing insemination efficiency and reducing costs in animal husbandry, a vital sector for global food production. Precise estrus period determination is essential to avoid economic losses, such as milk production reductions, delayed calf births, and disqualification from government support. The proposed method integrates estrus period detection with cow identification using augmented reality (AR). It initiates deep learning-based mounting detection, followed by identifying the mounting region of interest (ROI) using YOLOv5. The ROI is then cropped with padding, and cow ID detection is executed using YOLOv5 on the cropped ROI. The system subsequently records the identified cow IDs. The proposed system accurately detects mounting behavior with 99% accuracy, identifies the ROI where mounting occurs with 98% accuracy, and detects the mounting couple with 94% accuracy. The high success of all operations with the proposed system demonstrates its potential contribution to AR and artificial intelligence applications in livestock farming. © 2023 by the authors.