Evidence that proteosome inhibitors and chemical chaperones can rescue the activity of retinol dehydrogenase 12 mutant T49M
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چکیده
منابع مشابه
Chemical Chaperones Improve Protein Secretion and Rescue Mutant Factor VIII in Mice with Hemophilia A
Inefficient intracellular protein trafficking is a critical issue in the pathogenesis of a variety of diseases and in recombinant protein production. Here we investigated the trafficking of factor VIII (FVIII), which is affected in the coagulation disorder hemophilia A. We hypothesized that chemical chaperones may be useful to enhance folding and processing of FVIII in recombinant protein produ...
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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...
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Missense mutations represent the most common cause of many genetic diseases including cystathionine beta-synthase (CBS) deficiency. Many of these mutations result in misfolded proteins, which lack biological function. The presence of chemical chaperones can sometimes alleviate or even restore protein folding and activity of mutant proteins. We present the purification and characterization of ei...
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مطالعه حاضر با هدف بررسی وجود رابطه ی ممکن میان هوشهای زبانی و منطقی زبان آموزان انگلیسی و تعداد و انواع مغلطه های زبانی و شواهد در متنهای استدلالی آنان انجام پذیرفته است.بدین منظور، 70 زبان آموز با سطح زبانی متوسط به بالا از دو موسسه زبان انگلیسی در ایران انتخاب شده و پرسشنامه های مزبوطه را تکمیل نموده و متنی استدلالی نوشتند. متون نوشته شده مورد بررسی قرار گرفته و نمرات پرسشنامه ها محاسبه شده ...
15 صفحه اولGenetic evidence that retinaldehyde dehydrogenase Raldh1 (Aldh1a1) functions downstream of alcohol dehydrogenase Adh1 in metabolism of retinol to retinoic acid.
Vitamin A (retinol) is a nutrient that is essential for developmental regulation but toxic in large amounts. Previous genetic studies have revealed that alcohol dehydrogenase Adh1 is required for efficient clearance of excess retinol to prevent toxicity, thus demonstrating that the mechanism involves oxidation of excess retinol to retinoic acid (RA). Whereas Adh1 plays a dominant role in the fi...
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ژورنال
عنوان ژورنال: Chemico-Biological Interactions
سال: 2011
ISSN: 0009-2797
DOI: 10.1016/j.cbi.2011.01.001