-
21
Crocetin Alleviates Inflammation in MPTP-Induced Parkinson’s Disease Models through Improving Mitochondrial Functions
Published 2020-01-01“…Our results showed that crocetin treatment attenuates MPTP-induced motor deficits and protects dopaminergic neurons. …”
Get full text
Article -
22
-
23
Whole transcriptome analysis of unmutated sporadic ALS patients' peripheral blood reveals phenotype-specific gene expression signature
Published 2025-03-01“…The work proposed stress the innovative view of ALS as a multi-systemic disorder rather than a pure motor neuron-associated and ‘neurocentric’ pathology. …”
Get full text
Article -
24
Safety and Efficacy of Nusinersen Focusing on Renal and Hematological Parameters in Spinal Muscular Atrophy
Published 2025-01-01“…ABSTRACT Background Spinal muscular atrophy (SMA) is a motor neuron disease caused by mutations in the SMN1 gene. …”
Get full text
Article -
25
M1 and M2 Functional Imprinting of Primary Microglia: Role of P2X7 Activation and miR-125b
Published 2016-01-01“…Mutations in over twenty genes associated with familial forms of ALS have provided insights into the mechanisms leading to motor neuron death. …”
Get full text
Article -
26
Identification of a metabolic brain network characterizing essential tremor
Published 2025-01-01“…Our findings suggest ET is characterized by an abnormal brain network associated with disease phenotype.…”
Get full text
Article -
27
A riboflavin-responsive neuronopathy with unique characteristics: Brown-Vialetto- Van Laere syndrome
Published 2022-06-01“…Sometimes the clinical spectrum mimics juvenile-onset motor neuron disease (MND). It is important to identify BVVLS that may respond to high doses of riboflavin. …”
Get full text
Article -
28
A riboflavin-responsive neuronopathy with unique characteristics: Brown-Vialetto- Van Laere syndrome
Published 2022-06-01“…Sometimes the clinical spectrum mimics juvenile-onset motor neuron disease (MND). It is important to identify BVVLS that may respond to high doses of riboflavin. …”
Get full text
Article -
29
Current Status of Treatment of Spinal and Bulbar Muscular Atrophy
Published 2012-01-01“…Spinal and bulbar muscular atrophy (SBMA) is the first member identified among polyglutamine diseases characterized by slowly progressive muscle weakness and atrophy of the bulbar, facial, and limb muscles pathologically associated with motor neuron loss in the spinal cord and brainstem. …”
Get full text
Article -
30
Widespread Striatal Delivery of GDNF from Encapsulated Cells Prevents the Anatomical and Functional Consequences of Excitotoxicity
Published 2019-01-01“…Animals were evaluated using a battery of validated motor tests, and histology was performed to determine the extent of GDNF diffusion and associated prevention of neuronal cell loss and behavioral deficits. …”
Get full text
Article -
31
In vivo mapping of sodium homeostasis disturbances in individual ALS patients: A brain 23Na MRI study.
Published 2025-01-01“…ALS patients with limited TSC increase had slower progression of disability or predominant lower motor neuron phenotype or shorter disease duration.…”
Get full text
Article -
32
Heterozygous CDKL5 Knockout Female Mice Are a Valuable Animal Model for CDKL5 Disorder
Published 2018-01-01“…These defects are associated with neuroanatomical alterations, such as reduced dendritic arborization and spine density of hippocampal neurons. …”
Get full text
Article -
33
-
34
Guillain-Barré syndrome following falciparum malaria infection: a case report
Published 2025-01-01“…This patient’s neurological symptoms pointed to paralysis of the lower motor neurons. Guillain-Barré Syndrome is also suggested by elevated protein levels and a lack of cells in the cerebrospinal fluid. …”
Get full text
Article -
35
Clinical and molecular outcomes from the 5-Year natural history study of SSADH Deficiency, a model metabolic neurodevelopmental disorder
Published 2024-04-01“…While epilepsy and psychiatric behaviors increase in severity with age, communication abilities and motor function tend to improve. iPSCs, which were differentiated into GABAergic neurons, represent the first in vitro neuronal model of SSADHD and express the neuronal marker microtubule-associated protein 2 (MAP2), as well as GABA. …”
Get full text
Article -
36
The Role of FRMD7 in Idiopathic Infantile Nystagmus
Published 2012-01-01“…Currently 45 mutations within FRMD7 have been associated with IIN, confirming the importance of FRMD7 in the pathogenesis of the disease. …”
Get full text
Article -
37
Oligonucleotide-Based Therapy for FTD/ALS Caused by the C9orf72 Repeat Expansion: A Perspective
Published 2013-01-01“…Amyotrophic lateral sclerosis (ALS) is a progressive and lethal disease of motor neuron degeneration, leading to paralysis of voluntary muscles and death by respiratory failure within five years of onset. …”
Get full text
Article -
38
Effects of Intravenous Administration of Human Umbilical Cord Blood Stem Cells in 3-Acetylpyridine-Lesioned Rats
Published 2012-01-01“…Cerebellar ataxias include a heterogeneous group of infrequent diseases characterized by lack of motor coordination caused by disturbances in the cerebellum and its associated circuits. …”
Get full text
Article -
39
Functions of Fasciculation and Elongation Protein Zeta-1 (FEZ1) in the Brain
Published 2010-01-01Get full text
Article -
40
An automated platform for simultaneous, longitudinal analysis of engineered neuromuscular tissues for applications in neurotoxin potency testing
Published 2025-01-01“…We present a turnkey iPSC-based model of the NMJ employing channelrhodopsin-2 expression within the motor neuron (MN) population driving muscle contraction in response to blue light. …”
Get full text
Article