Defective RNA metabolism is an emerging mechanism involved in ALS pathogenesis and possibly in other neurodegenerative disorders. Here, we show that microRNA (miRNA) activity is essential for long-term survival of postmitotic spinal motor neurons (SMNs) in vivo. Thus, mice that do not process miRNA in SMNs exhibit hallmarks of spinal muscular atrophy (SMA), including sclerosis of the spinal cord ventral horns, aberrant end plate architecture, and myofiber atrophy with signs of denervation. Furthermore, a neurofilament heavy subunit previously implicated in motor neuron degeneration is specifically up-regulated in miRNA-deficient SMNs. We demonstrate that the heavy neurofilament subunit is a target of miR-9, a miRNA that is specifically down-regulated in a genetic model of SMA. These data provide evidence for miRNA function in SMN diseases and emphasize the potential role of miR-9-based regulatory mechanisms in adult neurons and neurodegenerative states.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2919953 | PMC |
| http://dx.doi.org/10.1073/pnas.1006151107 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
[Comprehensive rehabilitation of spinal trauma patients with extended involvement of nursing staff and neurometabolic therapy].
Zh Nevrol Psikhiatr Im S S Korsakova
December 2024
Russian University of Medicine, Moscow, Russia.
Objective: Analysis of the effectiveness of the use of the drug Cytoflavin and the organization of the activities of nursing staff, within the framework of nursing care, in the complex therapy of patients with spinal cord injury (PSMT).
Material And Methods: Material and methods. 40 patients with PSMT due to a gunshot wound were examined, who were divided into two equal groups depending on the type of therapy performed: group 1 patients received the full volume of stage I medical rehabilitation (with additional use of neurodevelopmental techniques under the supervision of a Bobata department nurse) and standard drug therapy, including a course of intravenous Cytoflavin infusions followed by tablet form; group 2 patients received the full volume of stage I medical rehabilitation and standard drug therapy, but did not receive Cytoflavin.
Spinally targeted paired associated stimulation with high-frequency peripheral component induces spinal level plasticity in healthy subjects.
Sci Rep
December 2024
BioMag Laboratory, HUS Diagnostic Center, Helsinki University Hospital, University of Helsinki and Aalto University School of Science, Helsinki, Finland.
A novel variant of paired-associative stimulation (PAS) consisting of high-frequency peripheral nerve stimulation (PNS) and high-intensity transcranial magnetic stimulation (TMS) above the motor cortex, called high-PAS, can lead to improved motor function in patients with incomplete spinal cord injury. In PAS, the interstimulus interval (ISI) between the PNS and TMS pulses plays a significant role in the location of the intended effect of the induced plastic changes. While conventional PAS protocols (single TMS pulse often applied with intensity close to resting motor threshold, and single PNS pulse) usually require precisely defined ISIs, high-PAS can induce plasticity at a wide range of ISIs and also in spite of small ISI errors, which is helpful in clinical settings where precise ISI determination can be challenging.
View Article and Find Full Text PDFCo-Existent Central and Peripheral Demyelination: Related or Coincidental?
Neurol Int
December 2024
Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA.
Background: Hereditary Sensory Motor Neuropathy (HSMN) 1A and Multiple Sclerosis (MS) are distinct demyelinating disorders affecting the peripheral and central nervous systems, respectively. We present a case of simultaneous occurrence of both conditions, exploring the clinical presentation, diagnostic workup, and potential interplay between these diseases. Case presentation and clinical approach: A 49-year-old male with a history of optic neuritis presented with progressive numbness, weakness, and sensory loss in all extremities over four years.
View Article and Find Full Text PDFThe Influence of Anesthesia on Neuromonitoring During Scoliosis Surgery: A Systematic Review.
NeuroSci
December 2024
Department of Palliative Medicine, Poznan University of Medical Sciences, 61-701 Poznań, Poland.
Background: Intraoperative neuromonitoring (IONM) is crucial for the safety of scoliosis surgery, providing real-time feedback on the spinal cord and nerve function, primarily through motor-evoked potentials (MEPs). The choice of anesthesia plays a crucial role in influencing the quality and reliability of these neuromonitoring signals. This systematic review evaluates how different anesthetic techniques-total intravenous anesthesia (TIVA), volatile anesthetics, and regional anesthesia approaches such as Erector Spinae Plane Block (ESPB), spinal, and epidural anesthesia-affect IONM during scoliosis surgery.
View Article and Find Full Text PDFDo adolescents with different types and degrees of idiopathic scoliosis curves differ in postural control compared to their healthy peers? a cross-sectional study.
BMC Musculoskelet Disord
December 2024
School of Health and Social Care, Department of Allied Health Professions, University of Teesside, Middlesbrough, UK.
Background: Numerous scoliosis research studies have investigated postural control changes in adolescents with idiopathic scoliosis and compared them to healthy controls. However, the results have been controversial. Therefore, the present study aimed to compare whether postural control in adolescent idiopathic scoliosis (AIS) patients is different from their age-matched healthy counterparts.
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!