Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[Motor neuron diseases. Present]

A Pou1

  • 1Servicio de Neurología, Hospital del Mar, Universidad Autónoma de Barcelona.

Neurologia (Barcelona, Spain)
|December 1, 1996
PubMed
Summary

Recent breakthroughs in motor neuron diseases like spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS) offer new insights. Advances in genetics, neurobiology, and neuroimmunology are paving the way for novel diagnostic and therapeutic strategies.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Distinct muscle imaging patterns in myofibrillar myopathies.

Neurology·2008
Same author

Dysferlin expression in monocytes: a source of mRNA for mutation analysis.

Neuromuscular disorders : NMD·2006
Same author

SMN2 copy number predicts acute or chronic spinal muscular atrophy but does not account for intrafamilial variability in siblings.

Journal of neurology·2005
Same author

Timing of chemoradiotherapy and patient selection for locally advanced nasopharyngeal carcinoma.

Clinical oncology (Royal College of Radiologists (Great Britain))·2003
Same author

[Angioplasty and stenting in severe stenosis of basilar artery refractory to pharmacotherapy].

Neurologia (Barcelona, Spain)·2003
Same author

[Sneddon syndrome presenting with dementia].

Neurologia (Barcelona, Spain)·2002

Area of Science:

  • Neurology and Genetics
  • Neurobiology and Neuroimmunology

Context:

  • Significant progress in understanding motor neuron diseases (MNDs), including spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS).
  • Focus on degenerative pathogenesis and historic advancements in the field.

Purpose:

  • To describe recent historic progress in understanding motor neuron diseases.
  • To highlight key areas of advancement: molecular genetics, neurobiology of neuron death, and neuroimmunology.
  • To emphasize diagnostic aspects, including electrophysiologic assessment and clinical features.

Summary:

  • Molecular genetics advances include discoveries of survival motor neurons (SMN) and neuronal apoptosis inhibitor protein (NAIP) in SMA, and the SOD1 gene in ALS.
  • Neurobiological insights into programmed cell death and the role of neurotrophic factors (e.g., NGF, BDNF) offer potential therapeutic targets.
  • Neuroimmunology highlights the significance of antiganglioside antibodies in diagnosing and managing neurogenic diseases like multifocal motor neuropathy and Guillain-Barré syndrome.

Impact:

  • These advancements are crucial for developing targeted therapeutic strategies to combat neuron degeneration in MNDs.
  • Improved understanding facilitates a comprehensive classification of SMA and better diagnostic approaches.
  • The course provides a summary of current knowledge in ten chapters on degenerative motor neuron diseases.

Related Experiment Videos