Results 31 to 40 of about 302,721 (262)

Motor neurone disease [PDF]

open access: yesPostgraduate Medical Journal, 2002
Abstract Motor neurone disease (MND), or amyotrophic lateral sclerosis (ALS), is a neurodegenerative disorder of unknown aetiology. Progressive motor weakness and bulbar dysfunction lead to premature death, usually from respiratory failure. Confirming the diagnosis may initially be difficult until the full clinical features are manifest.
openaire   +3 more sources

Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons. [PDF]

open access: yesPLoS ONE, 2012
TDP-43 pathology in spinal motor neurons is a neuropathological hallmark of sporadic amyotrophic lateral sclerosis (ALS) and has recently been shown to be closely associated with the downregulation of an RNA editing enzyme called adenosine deaminase ...
Takuto Hideyama   +4 more
doaj   +1 more source

Motor Band Sign in Motor Neuron Disease: A Marker for Upper Motor Neuron Involvement

open access: yesCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques, 2022
ABSTRACT:Background and Objective:The prevalence and role of the motor band sign (MBS) remain unclear in motor neuron disease. We report the frequency of MBS in amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS), its correlation with clinical upper motor neuron (UMN) signs, and prognostic value in ALS.Methods:We conducted a ...
Hee Sahng Chung   +3 more
openaire   +2 more sources

Functional Diversification of Motor Neuron-specific Isl1 Enhancers during Evolution.

open access: yesPLoS Genetics, 2015
Functional diversification of motor neurons has occurred in order to selectively control the movements of different body parts including head, trunk and limbs.
Namhee Kim   +3 more
doaj   +1 more source

Motor Neurone Disease [PDF]

open access: yesPostgraduate Medical Journal, 1962
THE story of motor neurone disease goes back more than a hundred years, to the latter half of the igth century, and the days of the great clinical neurologists of France. Of the many famous names linked to this story three are pre-eminent: Charcot-physician and neuropathologist, and great teacher, who became even more renowned for his studies of ...
openaire   +2 more sources

HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem Cells of Peripheral Neuropathy Patients with HSPB1 Mutation

open access: yesStem Cells International, 2016
The Charcot-Marie-Tooth disease 2F (CMT2F) and distal hereditary motor neuropathy 2B (dHMN2B) are caused by autosomal dominantly inherited mutations of the heat shock 27 kDa protein 1 (HSPB1) gene and there are no specific therapies available yet.
Ji-Yon Kim   +11 more
doaj   +1 more source

Glutamatergic cerebellar neurons differentially contribute to the acquisition of motor and social behaviors

open access: yesNature Communications, 2023
Insults to the developing cerebellum can cause motor, language, and social deficits. Here, we investigate whether developmental insults to different cerebellar neurons constrain the ability to acquire cerebellar-dependent behaviors. We perturb cerebellar
Meike E. van der Heijden   +6 more
doaj   +1 more source

Clinical and Biological Features of Response in Resistant Neuroblastoma to 131I‐Metaiodobenzylguanidine Radiotherapy in the Anti‐GD2 Immunotherapy Era

open access: yesPediatric Blood &Cancer, EarlyView.
ABSTRACT Background 131I‐metaiodobenzylguanidine (131I‐MIBG) radiotherapy is a key treatment for relapsed and refractory (R/R) neuroblastoma (NB). Patients with R/R disease treated in the modern era are increasingly exposed to anti‐GD2 immunotherapy, which exerts selective pressure and may modify both tumor cell state and microenvironment.
Benjamin J. Lerman   +7 more
wiley   +1 more source

Increased Risk of Sarcomas in Children With Congenital Anomalies: Findings From the Genetic Overlap Between Anomalies and Cancer in Kids (GOBACK) Registry Linkage Study

open access: yesPediatric Blood &Cancer, EarlyView.
ABSTRACT Background Pediatric sarcomas are a heterogeneous group of tumors that contribute disproportionately to cancer mortality in children. Although congenital anomalies are among the strongest known risk factors for childhood cancer, the risk of specific sarcoma subtypes among affected individuals has not yet been thoroughly evaluated. Procedure We
Russ Wolters   +17 more
wiley   +1 more source

GAL4 Drivers Specific for Type Ib and Type Is Motor Neurons in Drosophila

open access: yesG3: Genes, Genomes, Genetics, 2019
The Drosophila melanogaster larval neuromuscular system is extensively used by researchers to study neuronal cell biology, and Drosophila glutamatergic motor neurons have become a major model system.
Juan J. Pérez-Moreno, Cahir J. O’Kane
doaj   +1 more source

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