CISD2, an ER BCL2-associated autophagy regulator also known as NAF-1, is responsible for the human degenerative disorder Wolfram Syndrome 2. In order to interrogate the physiological role of CISD2 we generated and characterized the Cisd2 gene deletion in mice. Cisd2 null mice manifest significant degeneration in skeletal muscle tissues, which is accompanied with augmented autophagy, dysregulated Ca ( 2+) homeostasis and elongated mitochondria. Our findings describe a novel role for BCL2-CISD2 in the homeostatic maintenance of skeletal muscle. It remains to be elucidated how and if the antagonism of the BECN1 autophagy-initiating complex and modulation of ER Ca ( 2+) homeostasis by BCL2-CISD2 are interconnected.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.4161/auto.20054 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Abnormality in field physical test predicts a reduced quadriceps strength in patients with hip- or knee-osteoarthritis. A prospective observational study.
PLoS One
December 2024
CHU Clermont Ferrand, Plateforme d'Exploration de la Mobilité, Pôle MOBEX, Université Clermont Auvergne, Clermont-Ferrand, France.
Background: In osteoarthritis quadriceps strength is an important outcome to assess exercise capacity and recovery after arthroplasty. However, its measurement is limited due to lack of time and the need for trained personnel and equipment whose accuracy is verified.
Objectives: To find out the determinants of a reduced quadriceps strength and to establish a score to screen for it.
Masseter muscle thickness before and after the correction of unilateral functional posterior crossbite in growing individuals: a prospective controlled clinical trial.
Eur J Orthod
December 2024
Division of Orthodontics, University Clinics of Dental Medicine, University of Geneva, 1 rue Michel-Servet, 1205 Geneva, Switzerland.
Objectives: To evaluate whether unilateral functional posterior crossbite in growing children creates an asymmetry in masseter muscle thickness and whether this asymmetry is normalized after crossbite correction.
Materials And Methods: Two groups of growing individuals were studied prospectively: (i) a treatment group: children with unilateral functional posterior crossbite, undergoing crossbite correction with maxillary expansion; and (ii) a control group: children without transversal malocclusions and orthodontic treatment. The thickness of the masseter muscles was measured bilaterally using ultrasonographic recordings at three time points: pre-treatment (T0); 9 months after (T1); and 30 months after posterior crossbite correction (T2); and at equivalent time points in the control group.
The Potential of Targeting APE1/Ref-1 as a Therapeutic Intervention for Duchenne Muscular Dystrophy.
Antioxid Redox Signal
December 2024
Department of Medicine-Western Health, Melbourne Medical School, The University of Melbourne, Parkville, Australia.
Inflammation and oxidative stress play crucial roles in the development and progression of skeletal muscle diseases. This review aims to examine the existing evidence regarding the involvement and inhibition of APE1/Ref-1 (apurinic/apyrimidinic endonuclease 1/redox factor 1) in diseases, then extrapolate this evidence to the context of skeletal muscle and discuss the potential beneficial effects of APE1/Ref-1 inhibition in ameliorating myopathy with a particular focus on dystrophic pathology. Currently, therapeutic interventions targeting pathways, such as nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB) and nuclear factor erythroid 2-related factor 2 (NRF2), have shown limited efficacy in both clinical and preclinical settings.
View Article and Find Full Text PDFExploring Gene Expression and Alternative Splicing in Duck Embryonic Myoblasts via Full-Length Transcriptome Sequencing.
Vet Sci
November 2024
College of Animal Science and Technology, Jiangxi Agricultural University, Nanchang 330045, China.
The duck industry is vital for supplying high-quality protein, making research into the development of duck skeletal muscle critical for improving meat and egg production. In this study, we leveraged Oxford Nanopore Technologies (ONT) sequencing to perform full-length transcriptome sequencing of myoblasts harvested from the leg muscles of duck embryos at embryonic day 13 (E13), specifically examining both the proliferative (GM) and differentiation (DM) phases. Our analysis identified a total of 5797 novel transcripts along with 2332 long non-coding RNAs (lncRNAs), revealing substantial changes in gene expression linked to muscle development.
View Article and Find Full Text PDFStable Convergent Polyneuronal Innervation and Altered Synapse Elimination in Muscles from Patients with Blepharospasm Responding Poorly to Recurrent Botulinum Type-A Neurotoxin Injections.
Toxins (Basel)
November 2024
Institut des Neurosciences Paris-Saclay, UMR 9197, CNRS/Université Paris-Sud, 91198 Gif-sur-Yvette, Cedex, France.
Botulinum neurotoxin type-A (BoNT/A), which blocks quantal acetylcholine (ACh) release at the neuromuscular junction (NMJ), has demonstrated its efficacy in the symptomatic treatment of blepharospasm. In 3.89% of patients treated for blepharospasm at Tenon Hospital, BoNT/A was no longer effective in relieving the patient's symptoms, and a partial upper myectomy of the muscle was performed.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!