Neuromuscular diseases due to chaperone mutations: a review and some new results
J Sarparanta, PH Jonson, S Kawan, B Udd - International journal of …, 2020 - mdpi.com
Skeletal muscle and the nervous system depend on efficient protein quality control, and they
express chaperones and cochaperones at high levels to maintain protein homeostasis …
express chaperones and cochaperones at high levels to maintain protein homeostasis …
Desmin-related cardiomyopathy: an unfolding story
PM McLendon, J Robbins - American Journal of Physiology …, 2011 - journals.physiology.org
The intermediate filament protein desmin is an integral component of the cardiomyocyte and
serves to maintain the overall structure and cytoskeletal organization within striated muscle …
serves to maintain the overall structure and cytoskeletal organization within striated muscle …
Enhanced autophagy ameliorates cardiac proteinopathy
MS Bhuiyan, JS Pattison, H Osinska… - The Journal of …, 2013 - Am Soc Clin Investig
Basal autophagy is a crucial mechanism in cellular homeostasis, underlying both normal
cellular recycling and the clearance of damaged or misfolded proteins, organelles and …
cellular recycling and the clearance of damaged or misfolded proteins, organelles and …
Diminished autophagy limits cardiac injury in mouse models of type 1 diabetes
X Xu, S Kobayashi, K Chen, D Timm, P Volden… - Journal of Biological …, 2013 - ASBMB
Cardiac autophagy is inhibited in type 1 diabetes. However, it remains unknown if the
reduced autophagy contributes to the pathogenesis of diabetic cardiomyopathy. We …
reduced autophagy contributes to the pathogenesis of diabetic cardiomyopathy. We …
Oxidation of myofibrillar proteins in human heart failure
M Canton, S Menazza, FL Sheeran… - Journal of the American …, 2011 - jacc.org
Objectives: We investigated the incidence and contribution of the oxidation/nitrosylation of
tropomyosin and actin to the contractile impairment and cardiomyocyte injury occurring in …
tropomyosin and actin to the contractile impairment and cardiomyocyte injury occurring in …
Identification of atrogin-1-targeted proteins during the myostatin-induced skeletal muscle wasting
S Lokireddy, IW Wijesoma, SK Sze… - … of Physiology-Cell …, 2012 - journals.physiology.org
Atrogin-1, a muscle-specific E3 ligase, targets MyoD for degradation through the ubiquitin-
proteasome-mediated system. Myostatin, a member of the transforming growth factor-β …
proteasome-mediated system. Myostatin, a member of the transforming growth factor-β …
Sustained activation of nuclear erythroid 2-related factor 2/antioxidant response element signaling promotes reductive stress in the human mutant protein aggregation …
NS Rajasekaran, S Varadharaj… - Antioxidants & redox …, 2011 - liebertpub.com
Inheritable missense mutations in small molecular weight heat-shock proteins (HSP) with
chaperone-like properties promote self-oligomerization, protein aggregation, and pathologic …
chaperone-like properties promote self-oligomerization, protein aggregation, and pathologic …
[HTML][HTML] The role of αB-crystallin in skeletal and cardiac muscle tissues
I Dimauro, A Antonioni, N Mercatelli… - Cell Stress and …, 2018 - Elsevier
All organisms and cells respond to various stress conditions such as environmental,
metabolic, or pathophysiological stress by generally upregulating, among others, the …
metabolic, or pathophysiological stress by generally upregulating, among others, the …
S14-phosphorylated rpn6 mediates proteasome activation by pka and alleviates proteinopathy
L Yang, N Parajuli, P Wu, J Liu, X Wang - Circulation Research, 2023 - Am Heart Assoc
BACKGROUND: A better understanding of the regulation of proteasome activities can
facilitate the search for new therapeutic strategies. A cell culture study shows that PKA …
facilitate the search for new therapeutic strategies. A cell culture study shows that PKA …
[HTML][HTML] Mitochondrial membrane potential is regulated by vimentin intermediate filaments
IS Chernoivanenko, EA Matveeva, VI Gelfand… - The FASEB …, 2015 - ncbi.nlm.nih.gov
This study demonstrates that the association of mitochondria with vimentin intermediate
filaments (VIFs) measurably increases their membrane potential. This increase is detected …
filaments (VIFs) measurably increases their membrane potential. This increase is detected …