Wilson's disease (WD) is an autosomal recessive disorder in which copper metabolism is impaired. In fact, copper accumulates in various organs and tissues can be seen and causes toxic effects in various tissues including liver, brain, kidneys and eyes. Sulfur amino acid is a metabolite of D-penicillamine and penicillamine and copper Chlator is a factor that causes urinary excretion of copper and WD therapeutic agent as well. The interesting thing about the neurological symptoms of Wilson's disease with penicillamine is the drug may worsen or even in an asymptomatic patient, the treatment may be creating symptoms. DTPA is a pentavalent compound containing carboxylic DTPA is a chemical compound that is used in radiation therapy and MRI. It can give the metal chelate with iron, copper and other cations can be conjugated and also treatment of internal body pollution caused by various elements, including raDOIactive elements. DTPA could not be lonely absorbed by the cell. The goal is to conjugate it with the G2 Dendrimer (Nanosized anionic linear biocompatible polymer) to bring it to the nano size and increase the intracellular uptake compared to the ground state. Based on the hypothesis, nanoconjugated DTPA-Dendrimer G2 was synthesized and then evaluated on Hep G2 WD cell model in vitro and the results showed a good effectiveness without any toxicity for the conjugate in decreasing the intracellular copper level comparing to gold standard D-penicillamine respectively. Based on the findings the nanosized conjugate seems to have very good prognoses and clinical future and this needs to be further investigated.
Backgrond: Muscular dystrophies consist of a number of juvenile and adult forms of complex disorders which generally cause weakness or efficiency defects affecting skeletal muscles or, in some kinds, other types of tissues in all parts of the body are vastly affected. In previous studies, it was observed that along with muscular dystrophy, immune inflammation was caused by inflammatory cells invasion -like T lymphocyte markers (CD8+/CD4+). Inflammatory processes play a major part in muscular fibrosis in muscular dystrophy patients. Additionally, a significant decrease in amounts of two myogenic recovery factors (myogenic differentation 1 [MyoD] and myogenin) in animal models was observed. The drug glatiramer acetate causes anti-inflammatory cytokines to increase and T helper (Th) cells to induce, in an as yet unknown mechanism. MyoD recovery activity in muscular cells justifies using it alongside this drug. Methods: In this study, a nanolipodendrosome carrier as a drug delivery system was designed. The purpose of the system was to maximize the delivery and efficiency of the two drug factors, MyoD and myogenin, and introduce them as novel therapeutic agents in muscular dystrophy phenotypic mice. The generation of new muscular cells was analyzed in SW1 mice. Then, immune system changes and probable side effects after injecting the nanodrug formulations were investigated. Results: The loaded lipodendrimer nanocarrier with the candidate drug, in comparison with the nandrolone control drug, caused a significant increase in muscular mass, a reduction in CD4+/CD8+ inflammation markers, and no significant toxicity was observed. The results support the hypothesis that the nanolipodendrimer containing the two candidate drugs will probably be an efficient means to ameliorate muscular degeneration, and warrants further investigation.
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