Publications by authors named "D Braconi"
Article Synopsis
- Alkaptonuria (AKU) is a genetic disorder that leads to degradation of connective tissues, causing issues like cartilage damage, tendon calcification, and early-onset osteoarthritis due to the build-up of homogentisic acid (HGA).
- HGA is reactive and promotes oxidative stress and inflammation, resulting in widespread, irreversible damage to the body.
- The review discusses inflammation and oxidative stress in AKU, explores the molecular mechanisms involving HGA, and introduces ApreciseKUre, a digital platform for analyzing AKU-related data.
Article Synopsis
- - Alkaptonuria is a rare genetic disorder caused by a lack of an enzyme that leads to the buildup of homogentisic acid, resulting in symptoms like dark urine, joint pain, and skin discoloration, particularly affecting connective tissues.
- - The disease usually shows symptoms around a person's 30s, but a diagnosis can be delayed due to vague symptoms and low physician awareness; management requires a team of specialists to monitor and treat the condition effectively.
- - Current research aims to enhance patient care and develop new treatment methods, focusing on personalized medicine to improve outcomes for individuals with alkaptonuria.
Nitisinone (NTBC) was recently approved to treat alkaptonuria (AKU), but there is no information on its impact on oxidative stress and inflammation, which are observed in AKU. Therefore, serum samples collected during the clinical studies SONIA1 (40 AKU patients) and SONIA2 (138 AKU patients) were tested for Serum Amyloid A (SAA), CRP and IL-8 by ELISA; Advanced Oxidation Protein Products (AOPP) by spectrophotometry; and protein carbonyls by Western blot. Our results show that NTBC had no significant effects on the tested markers except for a slight but statistically significant effect for NTBC, but not for the combination of time and NTBC, on SAA levels in SONIA2 patients.
View Article and Find Full Text PDFThe serendipitous discovery of the HPPD inhibitors from allelopathic plants opened the way for searching new and effective herbicidal agents by application of classical hit-to-lead optimization approaches. A plethora of active and selective compounds were discovered that belong to three major classes of cyclohexane-based triketones, pyrazole-based diketones, and diketonitriles. In addition, to enhance inhibitory constant and herbicidal activity, many efforts were also made to gain broader weed control, crop safety, and eventual agricultural applicability.
View Article and Find Full Text PDFAlkaptonuria (AKU) is an ultra-rare genetic disease caused by a deficient activity of the enzyme homogentisate 1,2-dioxygenase (HGD) leading to the accumulation of homogentisic acid (HGA) on connective tissues. Even though AKU is a multi-systemic disease, osteoarticular cartilage is the most affected system and the most damaged tissue by the disease. In chondrocytes, HGA causes oxidative stress dysfunctions, which induce a series of not fully characterized cellular responses.
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