Results 61 to 70 of about 477,265 (189)
Ambroxol as a pharmacological chaperone for mutant glucocerebrosidase.
Blood Cells Mol Dis, 2013 Gaucher disease (GD) is characterized by accumulation of glucosylceramide in lysosomes due to mutations in the GBA1 gene encoding the lysosomal hydrolase β-glucocerebrosidase (GCase). The disease has a broad spectrum of phenotypes, which were divided into three different Types; Type 1 GD is not associated with primary neurological disease while Types 2
Bendikov-Bar I +3 more
europepmc +4 more sources
Cells, 2019 HSPA8/HSC70 is a molecular chaperone involved in a wide variety of cellular processes. It plays a crucial role in protein quality control, ensuring the correct folding and re-folding of selected proteins, and controlling the elimination of abnormally ...
Srinivasa Reddy Bonam +2 more
doaj +1 more source
Sigma-1 receptor modulation fine-tunes KV1.5 channels and impacts pulmonary vascular function
Pharmacological Research, 2023 KV1.5 channels are key players in the regulation of vascular tone and atrial excitability and their impairment is associated with cardiovascular diseases including pulmonary arterial hypertension (PAH) and atrial fibrillation (AF).
Alba Vera-Zambrano +13 more
doaj +1 more source
The Mitochondrial Chaperone Protein TRAP1 Mitigates α-Synuclein Toxicity [PDF]
, 2012 Overexpression or mutation of alpha-Synuclein is associated with protein aggregation and interferes with a number of cellular processes, including mitochondrial integrity and function.
Butler, Erin K. +41 more
core +2 more sources
Pteridines, 2017 We have developed a fluorescence assay system to monitor the protein levels of human phenylalanine hydroxylase (hPAH). Wild-type (WT) and three mutant hPAHs (I65T, L255V, and S349L) were expressed as green fluorescent protein (GFP)-tagged forms in a PAH ...
Kim Hye-Lim +2 more
doaj +1 more source
The structure of the PapD-PapGII pilin complex reveals an open and flexible P5 pocket [PDF]
, 2012 P pili are hairlike polymeric structures that mediate binding of uropathogenic Escherichia coli to the surface of the kidney via the PapG adhesin at their tips.
Pinkner, J. +16 more
core +1 more source
A Chemical Chaperone That Prevents Insulin Fibrillation [PDF]
, 2021 Insulin, a peptide hormone, is susceptible to amyloid formation upon exposure to aberrant physiological conditions, result-ing in a loss of its bioactivity.
Anirban, Das +2 more
core +1 more source
Characterization of the proteostasis roles of glycerol accumulation, protein degradation and protein synthesis during osmotic stress in C. elegans. [PDF]
PLoS ONE, 2012 Exposure of C. elegans to hypertonic stress-induced water loss causes rapid and widespread cellular protein damage. Survival in hypertonic environments depends critically on the ability of worm cells to detect and degrade misfolded and aggregated ...
Kristopher Burkewitz +4 more
doaj +1 more source
SLAS Discovery, 2023 The beta-glucocerebrosidase (GBA1) gene encodes the lysosomal beta-glucocerebrosidase (GCase) that metabolizes the lipids glucosylceramide (GlcCer) and glucosylsphingosine (GlcSph). Biallelic loss-of-function mutations in GBA1 such as L444P cause Gaucher
Yusuke Naito +5 more
doaj +1 more source
Nicotine is a Selective Pharmacological Chaperone of Acetylcholine Receptor Number and Stoichiometry. Implications for Drug Discovery [PDF]
, 2009 The acronym SePhaChARNS, for “selective pharmacological chaperoning of acetylcholine receptor number and stoichiometry,” is introduced. We hypothesize that SePhaChARNS underlies classical observations that chronic exposure to nicotine causes ...
Miwa, Julie +9 more
core +1 more source