Chemical Chaperones: A Pharmacological Strategy for Disorders of Protein Folding and Trafficking
نویسندگان
چکیده
منابع مشابه
Protein folding: Chaperones get Hip
In the cell, nascent and completed polypeptides may misfold and aggregate. A class of proteins called molecular chaperones has evolved to facilitate the production of native, functional forms of proteins. These chaperones also aid in the translocation of proteins across biological membranes. The ubiquitous heat-shock protein (Hsp) 70 class of chaperones has been the subject of intensive study o...
متن کاملMolecular chaperones--cellular machines for protein folding.
Proteins are linear polymers synthesized by ribosomes from activated amino acids. The product of this biosynthetic process is a polypeptide chain, which has to adopt the unique three-dimensional structure required for its function in the cell. In 1972, Christian Anfinsen was awarded the Nobel Prize for Chemistry for showing that this folding process is autonomous in that it does not require any...
متن کاملChaperones and protein folding in the archaea.
A survey of archaeal genomes for the presence of homologues of bacterial and eukaryotic chaperones reveals several interesting features. All archaea contain chaperonins, also known as Hsp60s (where Hsp is heat-shock protein). These are more similar to the type II chaperonins found in the eukaryotic cytosol than to the type I chaperonins found in bacteria, mitochondria and chloroplasts, although...
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Multidrug resistance protein 3 (MDR3, ABCB4) is a hepatocellular membrane protein that mediates biliary secretion of phosphatidylcholine. Null mutations in ABCB4 gene give rise to severe early-onset cholestatic liver disease. We have previously shown that the disease-associated mutations p.G68R, p.G228R, p.D459H, and p.A934T resulted in retention of ABCB4 in the endoplasmic reticulum, thus fail...
متن کاملDevelopment of a pharmacorphore model for pharmacological chaperones targeting mutant trafficking-deficient CNG channels
Complete colorblindness (achromatopsia) is caused by autosomal recessively inherited mutations in the retinal phototransduction pathway, predominantly in the CNGA3and CNGB3-subunit of the cyclic nucleotidegated (CNG) channels in cone photoreceptors. CNGA3, which is mutated in about 25% of the achromatopsia patients, mainly harbors missense mutations which frequently impair the folding and/or tr...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 2002
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-200212000-00004