Results 111 to 120 of about 1,305,355 (298)

Peroxisomal Proteostasis Involves a Lon Family Protein That Functions as Protease and Chaperone [PDF]

, 2012
Proteins are subject to continuous quality control for optimal proteostasis. The knowledge of peroxisome quality control systems is still in its infancy. Here we show that peroxisomes contain a member of the Lon family of proteases (Pln).
Kikhney, Alexey, Roermund, Carlo W.T. van,, van Roermund, C. W. T., Opalinski, L., Kikhney, A., Williams, Chris,, de Boer, R., Veenhuis, Marten, Kikhney, Alexey,, de Boer, Rinse, Williams, C., Williams, Chris, Opalinski, Lukasz, Bartoszewska, M., Klei, Ida J. van der,, van der Klei, Ida J., Boer, Rinse de,, Opaliński, Łukasz,, van der Klei, I. J., van Roermund, Carlo W. T., Veenhuis, Marten,, Veenhuis, M., van der Klei, Ida J.; id_orcid, Bartoszewska, Magdalena, Bartoszewska, Magdalena,   +24 more
core   +1 more source

Molecular Chaperones in Protein Quality Control

BMB Reports, 2005
Proteins must fold into their correct three-dimensional conformation in order to attain their biological function. Conversely, protein aggregation and misfolding are primary contributors to many devastating human diseases, such as prion-mediated infections, Alzheimer's disease, type II diabetes and cystic fibrosis.
Sukyeong, Lee, Francis T F, Tsai
openaire   +3 more sources

Linking neurogenesis, oligodendrogenesis, and myelination defects to neurodevelopmental disruption in primary mitochondrial disorders

FEBS Letters, EarlyView.
Mitochondrial remodeling shapes neural and glial lineage progression by matching metabolic supply with demand. Elevated OXPHOS supports differentiation and myelin formation, while myelin compaction lowers mitochondrial dependence, revealing mitochondria as key drivers of developmental energy adaptation.
Sahitya Ranjan Biswas, Porter L. Tomsick, Alicia M. Pickrell, Paul D. Morton   +3 more
wiley   +1 more source

An isoform of 14‐3‐3 protein regulates transbilayer lipid movement at the plasma membrane

FEBS Letters, EarlyView.
Loss of 14‐3‐3ζ in CHO cells confers resistance to exogenous phosphatidylserine (PS) and impairs endocytosis‐independent inward flip‐flop of fluorescent PS at the plasma membrane. RNAi‐mediated knockdown reproduces this defect, while no additive effect is seen in ATP11C‐deficient cells.
Akiko Yamaji‐Hasegawa, Akira Hattori, Masafumi Tsujimoto, Toshihide Kobayashi   +3 more
wiley   +1 more source

Towards the physiological role of ER quality control in an organismal context

, 2014
ER-associated degradation (ERAD) plays a pivotal role in cells with a highly active secretory apparatus, such as B lymphocytes, beta cells in the pancreas or hepatocytes.
König, P.A., Cellular Protein Chemistry
core  

Protein Quality Control and Metabolism: Bidirectional Control in the Heart

, 2015
The prevalence of heart disease, especially heart failure, continues to increase, and cardiovascular disease remains the leading cause of death worldwide.
Wang, Zhao V., Joseph A. Hill, Hill, Joseph A., Zhao V. Wang   +3 more
core   +1 more source

Protein quality control in the secretory pathway

Journal of Cell Biology, 2019
Protein folding is inherently error prone, especially in the endoplasmic reticulum (ER). Even with an elaborate network of molecular chaperones and protein folding facilitators, misfolding can occur quite frequently. To maintain protein homeostasis, eukaryotes have evolved a series of protein quality-control checkpoints.
Zhihao Sun, Jeffrey L. Brodsky
openaire   +2 more sources

The ubiquitin ligase RNF115 is required for the clearance of damaged lysosomes

FEBS Letters, EarlyView.
Upon lysosomal rupture, an E3 ubiquitin ligase RNF115 translocates from the cytosol to the damaged lysosomal membrane. Moreover, RNF115 depletion impairs the clearance of damaged lysosomes, identifying it as a key regulator of lysosomal quality control.
Sae Nakanaga, Toshiki Takahashi, Akiko Kuma, Hiroyuki Kawahara   +3 more
wiley   +1 more source

Protein Quality Control Activation and Microtubule Remodeling in Hypertrophic Cardiomyopathy

Cells, 2019
Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder. It is mainly caused by mutations in genes encoding sarcomere proteins. Mutant forms of these highly abundant proteins likely stress the protein quality control (PQC) system ...
Larissa M. Dorsch, Maike Schuldt, Cristobal G. dos Remedios, Arend F. L. Schinkel, Peter L. de Jong, Michelle Michels, Diederik W. D. Kuster, Bianca J. J. M. Brundel, Jolanda van der Velden   +8 more
doaj   +1 more source

Annual Report on External Quality Assessment in Urinalysis in Korea (2000) [PDF]

, 2001
Two External quality assesment trials which composed of eight control materials(eight chemical materials) for interlaboratory quality control assesment in urinalysis were performed with 456 participants in the year of 2000.
김정호
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