نتایج جستجو برای: chemical chaperone
تعداد نتایج: 390727 فیلتر نتایج به سال:
ACTIVE CBS CAN BE EXPRESSED IN HEME-FREE SYSTEMS IN THE PRESENCE OF METAL-SUBSTITUTED PORPHYRINS OR A CHEMICAL CHAPERONE. Tomas Majtan, Laishram R. Singh, Liqun Wang, Warren D. Kruger, and Jan P. Kraus* 1 Department of Pediatrics, University of Colorado Denver, 12800 E 19th Ave, Aurora, CO 80045, USA 2 Department of Genomics & Biotechnology, Institute of Molecular Biology SAS, Dubravska cesta 2...
Mucopolysaccharidosis type IIIB is a devastating neurological disease caused by lack of the lysosomal enzyme, α-N-acetylglucosaminidase (NAGLU), leading to toxic accumulation heparan sulfate. Herein we explored pharmacological chaperone approach enhance residual activity NAGLU in patient fibroblasts. Capitalizing on three-dimensional structures two modest homoiminosugar-based inhibitors complex...
We performed proteomic studies using the GRP78 chaperone-inhibitor drug AR-12 (OSU-03012) as bait. Multiple additional chaperone and chaperone-associated proteins were shown to interact with AR-12, including: GRP75, HSP75, BAG2; HSP27; ULK-1; and thioredoxin. AR-12 down-regulated in situ immuno-fluorescence detection of ATP binding chaperones using antibodies directed against the NH2-termini of...
The yeast MAPKKK Ste11 activates three MAP kinase pathways, including pheromone signaling, osmosensing, and pseudohyphal/invasive growth pathways. We identified two chemical compounds, BTB03006 and GK03225, that suppress growth defects induced by Ste11 activation in diploid yeast cells. BTB03006, but not GK03225, was found to suppress growth defects induced by both alpha-factor and Ste4 G(beta)...
Sepsis-induced lymphopenia is a major cause of morbidities in intensive care units and in populations with chronic conditions such as renal failure, diabetes, HIV and alcohol abuse. Currently, other than supportive care and antibiotics, there are no treatments for this condition. We developed an in vitro assay to understand the role of the ER-stress-mediated apoptosis process in lymphocyte deat...
The stress-inducible molecular chaperone, HSP72, is an important therapeutic target in oncology, but inhibiting this protein with small molecules has proven particularly challenging. Validating HSP72 inhibitors in cells is difficult owing to competition with the high affinity and abundance of its endogenous nucleotide substrates. We hypothesized this could be overcome using a cysteine-targeted ...
Tetrahydrobiopterin (BH4) supplementation in patients with BH4-responsive phenylalanine hydroxylase (PAH) deficiency is an alternative to low-phenylalanine diet. To further investigate hepatic BH4-responsiveness, oral administration of 50 mg BH4/kg/day for 5 weeks was performed in wild-type mice. We observed a 2-fold increase in PAH protein by quantitative Western blot analysis and a 1.7-fold i...
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