Results 161 to 170 of about 477,265 (189)
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Pharmacological chaperones: a new twist on receptor folding
Trends in Pharmacological Sciences, 2000Protein misfolding is at the root of several genetic human diseases. These diseases do not stem from mutations within the active domain of the proteins, but from mutations that disrupt their three-dimensional conformation, which leads to their intracellular retention by the quality control apparatus of the cell.
J P, Morello +3 more
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Chemical and pharmacological chaperones as new therapeutic agents
Expert Reviews in Molecular Medicine, 2007Proteins that are exported from the cell, or targeted to the cell surface or other organelles, are synthesised and assembled in the endoplasmic reticulum and then delivered to their destinations. Point mutations – the most common cause of human genetic diseases – can inhibit folding and assembly of the protein in the endoplasmic reticulum. The unstable
Tip W, Loo, David M, Clarke
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Designation of imidazole-containing dipeptides as pharmacological chaperones
Human & Experimental Toxicology, 2010We review the dichotomous regulatory roles of natural imidazole-containing peptidomimetics (N-acetylcarnosine [NAC], carcinine, non-hydrolized carnosine) in maintaining skin homeostasis that determines whether keratinocytes survive or undergo apoptosis in response to various insults and in the development of skin diseases. General strategies addressing
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Pharmacological Chaperoning in Fabry Disease
2011Fabry Disease is an X-‐linked lysosomal storage disorder characterized by a variety of symptoms including hypohydrosis, seizures, cardiac abnormalities, skin lesions, and chronic pain. These symptoms stem from a lack of functional endogenous α-‐ Galactosidase A (α-GAL), which leads to an accrual of its natural substrate.
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Cloramphenicol: A pharmacological chaperone?
Molecular Genetics and Metabolism, 2012Roberto Giugliani +6 more
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A pharmacological chaperone on the horizon
Nature Reviews Nephrology, 2016Martina Gaggl, Gere Sunder-Plassmann
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Advances in the Development of Pharmacological Chaperones for the Mucopolysaccharidoses
International Journal of Molecular Sciences, 2020Alexander Rodríguez-López +1 more
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