Distinct Changes in Synaptic Protein Composition at Neuromuscular Junctions of Extraocular Muscles versus Limb Muscles of ALS Donors

Liu, Jing-Xia; Brännström, Thomas; Andersen, Peter M.; Pedrosa-Domellöf, Fatima
February 2013
PLoS ONE;Feb2013, Vol. 8 Issue 2, p1
Academic Journal
The pathophysiology of amyotrophic lateral sclerosis (ALS) is very complex and still rather elusive but in recent years evidence of early involvement of the neuromuscular junctions (NMJs) has accumulated. We have recently reported that the human extraocular muscles (EOMs) are far less affected than limb muscles at the end-stage of ALS from the same donor. The present study aimed to compare the differences in synaptic protein composition at NMJ and in nerve fibers between EOM and limb muscles from ALS donors and controls. Neurofilament light subunit and synaptophysin decreased significantly at NMJs and in nerve fibers in limb muscles with ALS whereas they were maintained in ALS EOMs. S100B was significantly decreased at NMJs and in nerve fibers in both EOMs and limb muscles of ALS donors, but other markers confirmed the presence of terminal Schwann cells in these NMJs. p75 neurotrophin receptor was present in nerve fibers but absent at NMJs in ALS limb muscles. The EOMs were able to maintain the integrity of their NMJs to a very large extent until the end-stage of ALS, in contrast to the limb muscles. Changes in Ca2+ homeostasis, reflected by altered S100B distribution, might be involved in the breakdown of nerve-muscle contact at NMJs in ALS.


Related Articles

  • The wasting diseases.  // Nutrition Health Review: The Consumer's Medical Journal;Spring90, Issue 54, p2 

    Focuses on the degenerative diseases amyotrophic lateral sclerosis, muscular dystrophy and multiple sclerosis. Characteristics apparent in all three diseases; Genetic factors in the development of degenerative diseases; Symptoms and manifestations.

  • MDA leads effort to combine ALS drugs. Markley, Jennifer // Inside Tucson Business;4/15/96, Vol. 6 Issue 3, p1 

    Reports that the Muscular Dystrophy Association (MDA) is spearheading an effort to begin combination-drug trials to treat amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. Development of protocols to kick-start the best way to treat ALS; Riluzole drug; Animal model...

  • Pre- and postsynaptic changes in the neuromuscular junction in dystrophic mice. Pratt, Stephen J. P.; Valencia, Ana P.; Le, Gloribel K.; Shah, Sameer B.; Lovering, Richard M.; Rudolf, Rüdiger; Jin-Hong Shin // Frontiers in Physiology;Sep2015, p1 

    Duchenne muscular dystrophy (DMD) is a devastating neuromuscular disease in which weakness, increased susceptibility to muscle injury, and inadequate repair appear to underlie the pathology. While most attention has focused within the muscle fiber, we recently demonstrated in mdx mice (murine...

  • Shamrock mobiles lead fight against Muscular Dystrophy.  // Breckenridge American;2/23/2008, Vol. 120, p4 

    The article reports on the sale of colorful Shamrocks mobiles by local merchants in Texas to support the Muscular Dystrophy Association's quest to cure muscle-wasting disease. Local families affected by diseases such as Duchenne Muscular Dystrophy and Lou Gehrig's disease (ALS) are directly...

  • The role of oxidative stress in degeneration of the neuromuscular junction in amyotrophic lateral sclerosis. Pollari, Eveliina; Goldsteins, Gundars; Bart, Geneviève; Koistinaho, Jari; Giniatullin, Rashid // Frontiers in Cellular Neuroscience;May2014, Vol. 8, p1 

    Amyotrophic lateral sclerosis (ALS) is characterized by the progressive loss of motoneurons and degradation of the neuromuscular junctions (NMJ). Consistent with the dying-back hypothesis of motoneuron degeneration the decline in synaptic function initiates from the presynaptic terminals in ALS....

  • Former LeBoeuf Partner Honored At 'Wings Over Wall Street' Dinner Benefiting ALS Research.  // Insurance Advocate;11/4/2002, Vol. 113 Issue 42, p43 

    Reports the recognition received by Peter K. Demmerle at "wings Over Wall Street" benefiting the Muscular Dystrophy Association to fund research on amyotrophic lateral sclerosis in New York City. Educational attainment; Career history; Contact information about "Wings Over Wall Street".

  • Look At Us, We're Walking. RADER, RICK // Exceptional Parent;Jun2015, Vol. 45 Issue 6, p4 

    The article focuses on the annual Labor Day Muscular Dystrophy Association (MDA) telethon which lasted from 1956 until 2014, and the Amyotrophic Lateral Sclerosis (ALS) Ice Bucket Challenge in the U.S., aimed at raising funds for muscular dystrophy and ALS patients. Topics include the amounts...

  • WINGS OVER WALL STREET.  // Traders Magazine;Dec2008, Vol. 21 Issue 289, p100 

    Photographs of several stockbrokers and members of the Muscular Dystrophy Association's (MDAs)Wings Over Wall Street annual charity activities for the Amyotropic Lateral Sclerosis (ALS) patients are presented.

  • Teaching Reflections: Yoga at the End of Life. Ammidown, Bea // Yoga Therapy in Practice;Sep2005, Vol. 1 Issue 1, p14 

    The article reports on the author's Yoga sessions with a 52-year-old registered nurse who was diagnosed with muscular dystrophy in the form of Amyotrophic Lateral Sclerosis. The patient participated in small restorative group classes and the Yoga community spirit. She also took one-on-one...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics