The novel use of the CFTR corrector C17 in muscular dystrophy: Pharmacological profile and in vivo efficacy.

Sarcoglycanopathies are rare forms of severe muscular dystrophies currently without a therapy. Mutations in sarcoglycan (SG) genes cause the reduction or absence of the SG-complex, a tetramer located in the sarcolemma that plays a protective role during muscle contraction. Missense mutations in SGCA, which cause α-sarcoglycanopathy, otherwise known as LGMD2D/R3, lead to folding defective forms of α-SG that are discarded by the cell quality control.

Green and Innovative Extraction: Phenolic Profiles and Biological Activities of Underutilized Plant Extracts Using Pulsed Electric Fields and Maceration.

Underutilized plant species such as (Forssk.) Less., Pomel, and L.

Determination of collagen types and mineral contents in fish skin and collagen-containing skin-derived protein hydrolysates before and after simulated digestion.

An digestion model was established to characterize the types of collagens in skin of cod, white fish, and salmon as well as their collagen-containing skin-derived protein hydrolysates (CSPH) before and after digestion. Moreover, the mineral content and their bioaccessibility were evaluated. Finally, the presence of heavy metals was evaluated to assess the safety of these products.

Bioavailability study of OTA, ZEN, and AFB1 along with bioactive compounds from tiger nut beverage and its by-products.

Mycotoxins pose significant health risks due to their prevalence in food products and severe health implications, including carcinogenicity. This study investigates the bioavailability of mycotoxins aflatoxin B1 (AFB1), ochratoxin A (OTA), and zearalenone (ZEN) individually and combined, in the presence of identified polyphenols from tiger nut beverage (TNB) and tiger nut by-product (TNBP) using the in vitro model Caco-2 cells, which simulates the human intestinal barrier. The objective is to understand how bioactive compounds from TNBP can mitigate the effects of AFB1, OTA and ZEN (and their combination) by bioavailability interference, contributing to safer food products and innovative food safety strategies.

Efficacy of Cystic Fibrosis Transmembrane Regulator Corrector C17 in Beta-Sarcoglycanopathy-Assessment of Patient's Primary Myotubes.

Limb-girdle muscular dystrophy type 2E/R4 (LGMD2E/R4) is a rare disease that currently has no cure. It is caused by defects in the gene, mainly missense mutations, which cause the impairment of the sarcoglycan complex, membrane fragility, and progressive muscle degeneration. Here, we studied the fate of some β-sarcoglycan (β-SG) missense mutants, confirming that, like α-SG missense mutants, they are targeted for degradation through the ubiquitin-proteasome system.