Results 131 to 140 of about 120,291 (395)

Wearable and Implantable Devices for Continuous Monitoring of Muscle Physiological Activity: A Review

Advanced Science, EarlyView.
Recent advances in materials and device engineering enable continuous, real‐time monitoring of muscle activity via wearable and implantable systems. This review critically summarizes emerging technologies for tracking electrophysiological, biomechanical, and oxygenation signals, outlines fundamental principles, and highlights key challenges and ...
Zhengwei Liao, Ata Golparvar, Mohammad Javad Bathaei, Filipe Arroyo Cardoso, Clementine M. Boutry   +4 more
wiley   +1 more source

Factors modifying the course of spinal muscular atrophy 5q

Нервно-мышечные болезни
Proximal spinal muscular atrophy 5q (SMA 5q) is a severe autosomal recessive neuromuscular disease characterized by progressive symptoms of flaccid paralysis and muscular atrophy due to degeneration of α-motor neurons of the anterior horns of the spinal ...
M. A. Akhkiamova, O. A. Shchagina, A. V. Polyakov   +2 more
doaj   +1 more source

Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus [PDF]

, 2017
Spinal muscular atrophy (SMA) is caused by a homozygous deletion or mutation in the survival motor neuron 1 (SMN1) gene that leads to reduced levels of SMN protein resulting in degeneration of motor neurons (MNs).
Berciano, Maria T., Calderó i Pardo, Jordi, Lafarga, Miguel, Narcís, Oriol J., Piedrafita Llorens, Lídia, Riancho, Javier, Tapia, Olga, Tarabal Mostazo, Olga   +7 more
core   +2 more sources

Nanogel Integrated Zwitterionic Injectable Hydrogel with Sequential Drug‐Releasing Capability for the Programmable Repair of Spinal Cord Injury

Advanced Science, EarlyView.
A novel drug‐loaded D/P‐g‐PSB nanogel‐incorporated hydrogel by the electrostatic attraction‐driven self‐assembling process. Sequential drug releasing property (melatonin is released first by physical diffusion, and then ibuprofen is released as the charge shielding effect and hydrogel degradation). This ion‐sensitive hydrogel platform with sequentially
Zhijian Wei, Susu Huang, Wencan Zhang, Jiayao Wen, Xiaolong Zhou, Wei He, Xiaoye Yang, Haifeng Wang, Guangxi Zhai, Bin Shi, Lin Jin, Dachuan Wang, Shiqing Feng, Lei Ye   +13 more
wiley   +1 more source

REEP1 Accumulation Disrupts ER Integrity and Drives Spinal Motoneuron Degeneration in Distal Hereditary Motor Neuropathy

Advanced Science, EarlyView.
REEP1 contributes to endoplasmic reticulum (ER) shaping. Variants either cause cortical motoneuron degeneration and hereditary spastic paraplegia (HSP) or spinal motoneuron degeneration and distal hereditary motor neuropathy (dHMN). Knockout causes less complex ER structures and cortical motoneuron loss.
Andrea Bock, Mona Schurig, Miles Willoughby, Andrea Mirecki, Eric Seemann, Kateryna Lohachova, Istvan Katona, Sonnhild Mittag, Lutz Liebmann, Patricia Franzka, Mehdi Heidari Horestani, Mukhran Khundadze, Thorsten Mosler, Timothy Louie, Marianne de Visser, Marian A. J. Weterman, Michael Kiehntopf, Christian Beetz, Sandor Nietzsche, Otmar Huber, Joachim Weis, Michael M. Kessels, Ramachandra M. Bhaskara, Britta Qualmann, Ivan Đikić, Christian A. Hübner   +25 more
wiley   +1 more source

Case Report: Atypical motor development in a patient with the mosaic form of Down syndrome and spinal muscular atrophy type 2- long-term observation

Frontiers in Genetics
A boy is presented in whom Down Syndrome mosaicism and spinal muscular atrophy by overlapping clinical symptoms delayed the diagnosis and caused complicated motor development.
Ewa Gajewska, Jędrzej Fliciński, Magdalena Sobieska, Joanna Michalska, Marcin Żarowski, Barbara Steinborn   +5 more
doaj   +1 more source

Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA. [PDF]

, 2014
Macroautophagy (hereafter autophagy) is a key pathway in neurodegeneration. Despite protective actions, autophagy may contribute to neuron demise when dysregulated.
Batlevi, Yakup, Carromeu, Cassiano, Cortes, Constanza J, Frankowski, Harald, Garden, Gwenn A, Ivanov, Nishi, La Spada, Albert R, Le, Amy, Miranda, Helen C, Muotri, Alysson R, Sopher, Bryce L, Young, Jessica E   +11 more
core  

Parabiosis, Assembloids, Organoids (PAO)

Advanced Science, EarlyView.
This review evaluates parabiosis, organoids, and assembloids as complementary disease models spanning systemic, organ, and multi‐organ levels. It highlights their construction strategies, applications, and current limitations, while emphasizing their integration with frontier technologies such as artificial intelligence, organ‐on‐a‐chip, CRISPR, and ...
Yang Hong, Lavonda Li, Lijie Yan, Long Bai, Jiacan Su, Xingcai Zhang   +5 more
wiley   +1 more source

Associations between Biomarkers, Phenotypes, and Motor Milestones in Chinese Patients with Spinal Muscular Atrophy Types 1-3

, 2023
Shijia Ouyang, Xiaoyin Peng, Jinli Bai, Wen‐Chen Huang, Hong Wang, Yu-wei Jin, Hui Jiao, Maoti Wei, Xiushan Ge, Fang Song, Yu-jin Qu   +10 more
openalex   +1 more source

CMAP changes upon symptom onset and during treatment in spinal muscular atrophy patients: lessons learned from newborn screening [PDF]

, 2020
Wen‐Chin Weng, Yu-Kan Hsu, Fu-Man Chang, Chun‐Yen Lin, Wuh‐Liang Hwu, Wang‐Tso Lee, Ni‐Chung Lee, Yin‐Hsiu Chien   +7 more
openalex   +1 more source