Results 71 to 80 of about 62,588 (334)

Prion degradation pathways: Potential for therapeutic intervention [PDF]

open access: yes, 2015
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the misfolding of native cellular PrP(C) into the disease-associated form PrP(Sc) that accumulates in the brain as disease progresses. Although treatments have yet to be
Goold, R, McKinnon, C, Tabrizi, SJ
core   +1 more source

VPS34 K29/K48 branched ubiquitination governed by UBE3C and TRABID regulates autophagy, proteostasis and liver metabolism

open access: yesNature Communications, 2021
The ubiquitin–proteasome system (UPS) and autophagy are two major quality control processes whose impairment is linked to a wide variety of diseases. The coordination between UPS and autophagy remains incompletely understood. Here, we show that ubiquitin
Yu-Hsuan Chen   +12 more
semanticscholar   +1 more source

Proteostasis Perturbations and Their Roles in Causing Sterile Inflammation and Autoinflammatory Diseases

open access: yesCells, 2022
Proteostasis, a portmanteau of the words protein and homeostasis, refers to the ability of eukaryotic cells to maintain a stable proteome by acting on protein synthesis, quality control and/or degradation.
Jonas Johannes Papendorf   +2 more
doaj   +1 more source

Enhancing mitochondrial proteostasis reduces amyloid-β proteotoxicity

open access: yesNature, 2017
Alzheimer’s disease is a common and devastating disease characterized by aggregation of the amyloid-β peptide. However, we know relatively little about the underlying molecular mechanisms or how to treat patients with Alzheimer’s disease. Here we provide
Vincenzo Sorrentino   +11 more
semanticscholar   +1 more source

CD90/Thy-1, a Cancer-Associated Cell Surface Signaling Molecule

open access: yesFrontiers in Cell and Developmental Biology, 2019
CD90 is a membrane GPI-anchored protein with one Ig V-type superfamily domain that was initially described in mouse T cells. Besides the specific expression pattern and functions of CD90 that were described in normal tissues, i.e., neurons, fibroblasts ...
Chloé Sauzay   +11 more
doaj   +1 more source

Proteostasis collapse is a driver of cell aging and death. [PDF]

open access: yes, 2019
What molecular processes drive cell aging and death? Here, we model how proteostasis-i.e., the folding, chaperoning, and maintenance of protein function-collapses with age from slowed translation and cumulative oxidative damage.
de Graff, Adam MR   +2 more
core  

HAX1-dependent control of mitochondrial proteostasis governs neutrophil granulocyte differentiation

open access: yesJournal of Clinical Investigation, 2022
The relevance of molecular mechanisms governing mitochondrial proteostasis to the differentiation and function of hematopoietic and immune cells is largely elusive. Through dissection of the network of proteins related to HCLS1-associated protein X-1, we
Yanxin Fan   +16 more
semanticscholar   +1 more source

Crosstalk between the ribosome quality control‐associated E3 ubiquitin ligases LTN1 and RNF10

open access: yesFEBS Letters, EarlyView.
Loss of the E3 ligase LTN1, the ubiquitin‐like modifier UFM1, or the deubiquitinating enzyme UFSP2 disrupts endoplasmic reticulum–ribosome quality control (ER‐RQC), a pathway that removes stalled ribosomes and faulty proteins. This disruption may trigger a compensatory response to ER‐RQC defects, including increased expression of the E3 ligase RNF10 ...
Yuxi Huang   +8 more
wiley   +1 more source

Proteostasis and Neuroinflammation in Alzheimer’s Disease

open access: yesJournal of Integrative Neuroscience
Alzheimer’s disease (AD) is the most common cause of dementia in older adults, marked by a gradual and irreversible deterioration of cognitive abilities, including memory and thinking skills. AD is highly heterogeneous, with variations in amyloid and tau
Augustina Potokiri, Hongmin Wang
doaj   +1 more source

Extracellular Chaperones and Proteostasis

open access: yesAnnual Review of Biochemistry, 2013
There exists a family of currently untreatable, serious human diseases that arise from the inappropriate misfolding and aggregation of extracellular proteins. At present our understanding of mechanisms that operate to maintain proteostasis in extracellular body fluids is limited, but it has significantly advanced with the discovery of a small but ...
Wyatt, Amy R   +3 more
openaire   +4 more sources

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