Scopus İndeksli Yayınlar Koleksiyonu / Scopus Indexed Publications Collection

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Access Right: Closed AccessPublisher: search.filters.publisher.Multidisciplinary Digital Publishing Institute (MDPI)

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  • Article
    Citation - Scopus: 1
    Assessing the Density of Wood in Heritage Buildings’ Elements Through Expedited Semi-Destructive Techniques
    (Multidisciplinary Digital Publishing Institute (MDPI), 2025) Henriques, Dulce F.; Sen, Ali Umut; Gomes, Maria da Gloria
    Featured Application The research presented and justified in this text aims to provide agents involved in the conservation and refurbishment of old timber elements in buildings with a correlation table for interpreting the readings obtained with penetration resistance testing equipment.Abstract Historically, wood has been among the main materials used in heritage buildings. However, the species and mechanical properties of these elements are often unknown. This uncertainty complicates safety assessment calculations, aggravated by the natural variability of the wood properties. The aim of this work is to assess the density of wooden elements in service using semi-destructive techniques that retain the integrity of structural elements. This research had two phases. First, penetration resistance tests were carried out on laboratory scale on Pinus sylvestris L. wood samples taken from 18th, 19th, and 20th century heritage buildings in Lisbon, Portugal. Later, a field study was carried out on wooden elements from the same buildings, involving needle penetration, core drilling, and moisture content determination tests. The laboratory test results showed a strong correlation between the needle penetration depth and wood density, with an R2 value of 0.76. The results of the field study indicated that the density estimated by the needle penetration test correlated effectively with the measured density of extracted cores after moisture correction, with an R2 of 0.99. In conclusion, the experimental results confirm that penetration resistance and moisture tests are reliable and practical for estimating wood density under in-service conditions.
  • 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.