Neuromuscular Junction

Publication Title: 
European journal of human genetics: EJHG

Spinal muscular atrophy (SMA) is the leading genetic cause of early childhood death worldwide and no therapy is available today. Many drugs, especially histone deacetylase inhibitors (HDACi), increase SMN levels. As all HDACi tested so far only mildly ameliorate the SMA phenotype or are unsuitable for use in humans, there is still need to identify more potent drugs. Here, we assessed the therapeutic power of the pan-HDACi JNJ-26481585 for SMA, which is currently used in various clinical cancer trials.

Author(s): 
Schreml, Julia
Riessland, Markus
Paterno, Mario
Garbes, Lutz
Roflbach, Kristina
Ackermann, Bastian
Kr‰mer, Jan
Somers, Eilidh
Parson, Simon H.
Heller, Raoul
Berkessel, Albrecht
Sterner-Kock, Anja
Wirth, Brunhilde
Publication Title: 
Human Molecular Genetics

In amyotrophic lateral sclerosis (ALS), the progressive loss of motor neurons is accompanied by extensive muscle denervation, resulting in paralysis and ultimately death. Upregulation of amyloid beta (A4) precursor protein (APP) in muscle fibres coincides with symptom onset in both sporadic ALS patients and the SOD1(G93A) mouse model of familial ALS.

Author(s): 
Bryson, J. Barney
Hobbs, Carl
Parsons, Michael J.
Bosch, Karen D.
Pandraud, Amelie
Walsh, Frank S.
Doherty, Patrick
Greensmith, Linda
Publication Title: 
PloS One

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that causes progressive paralysis due to motor neuron death. Several lines of published evidence suggested that inhibition of epidermal growth factor receptor (EGFR) signaling might protect neurons from degeneration. To test this hypothesis in vivo, we treated the SOD1 transgenic mouse model of ALS with erlotinib, an EGFR inhibitor clinically approved for oncology indications.

Author(s): 
Le Pichon, Claire E.
Dominguez, Sara L.
Solanoy, Hilda
Ngu, Hai
Lewin-Koh, Nicholas
Chen, Mark
Eastham-Anderson, Jeffrey
Watts, Ryan
Scearce-Levie, Kimberly
Publication Title: 
Molecular Therapy: The Journal of the American Society of Gene Therapy

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive loss of motor neurons in the brain and spinal cord. We have recently shown that human mesenchymal stem cells (hMSCs) modified to release glial cell line-derived neurotrophic factor (GDNF) decrease disease progression in a rat model of ALS when delivered to skeletal muscle. In the current study, we determined whether or not this effect could be enhanced by delivering GDNF in concert with other trophic factors.

Author(s): 
Krakora, Dan
Mulcrone, Patrick
Meyer, Michael
Lewis, Christina
Bernau, Ksenija
Gowing, Genevieve
Zimprich, Chad
Aebischer, Patrick
Svendsen, Clive N.
Suzuki, Masatoshi
Publication Title: 
Nihon Yakurigaku Zasshi. Folia Pharmacologica Japonica

The effect of oxatomide, an antiallergic drug, on the central and peripheral nervous systems were investigated, and the following results were obtained: Oxatomide at oral doses of 30-100 mg/kg produced little or no effect on the spontaneous and cooperative movements in mice, hexobarbital-induced hypnosis in mice, body temperature in rats, and did not induce muscle relaxation, the analgesic effect, anticonvulsive effects and anti-physostigmine effect. Oxatomide at doses of 300 mg/kg or more produced sedation followed by an increase in the responses to stimuli in mice and rats.

Author(s): 
Ohmori, K.
Ishii, H.
Shuto, K.
Nakamizo, N.
Publication Title: 
Anaesthesiologie Und Reanimation

Calcium entry blockers are now widely employed in the treatment of cardiovascular diseases and perioperative hypertension. In patients with coronary heart disease nifedipine therapy should be continued perioperatively to avoid coronary artery spasm. Animal experiments have demonstrated that calcium entry blockers potentiate the neuromuscular blockade induced by nondepolarizing blocking agents. In patients, an atracurium-induced neuromuscular depression is prolonged by intravenous nifedipine.

Author(s): 
Jelen-Esselborn, S.
Blobner, M.
Hölzl, J.
Felber, A. R.
Publication Title: 
Scandinavian Journal of Medicine & Science in Sports

The use of cryotherapy, i.e. the application of cold for the treatment of injury or disease, is widespread in sports medicine today. It is an established method when treating acute soft tissue injuries, but there is a discrepancy between the scientific basis for cryotherapy and clinical studies. Various methods such as ice packs, ice towels, ice massage, gel packs, refrigerant gases and inflatable splints can be used. Cold is also used to reduce the recovery time as part of the rehabilitation programme both after acute injuries and in the treatment of chronic injuries.

Author(s): 
Swenson, C.
Swärd, L.
Karlsson, J.
Publication Title: 
Annals of Anatomy = Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft

Insufficient recovery after peripheral nerve injury has been attributed to (i) poor pathfinding of regrowing axons, (ii) excessive collateral axonal branching at the lesion site and (iii) polyneuronal innervation of the neuromuscular junctions (NMJ). The facial nerve transection model has been used initially to measure restoration of function after varying therapies and to examine the mechanisms underlying their effects. Since it is very difficult to control the navigation of several thousand axons, efforts concentrated on collateral branching and NMJ-polyinnervation.

Author(s): 
Skouras, Emmanouil
Ozsoy, Umut
Sarikcioglu, Levent
Angelov, Doychin N.
Publication Title: 
Journal of Neuropathology and Experimental Neurology

A large fraction of hereditary demyelinating neuropathies, classified as Charcot-Marie-Tooth disease type 1A, is associated with misexpression of peripheral myelin protein 22. In this study, we characterized morphologic and biochemical changes that occur with diseaseprogression in neuromuscular tissue of Trembler-J mice, a spontaneous rodent model of Charcot-Marie-Tooth disease type 1A. Using age-matched, 2- and 10-month-old, wild-type and Trembler-J mice, we observed neuromuscular deficits that progress from distal to proximal regions.

Author(s): 
Nicks, Jessica Renee
Lee, Sooyeon
Kostamo, Kathryne Ann
Harris, Andrew Benford
Sookdeo, Amanda M.
Notterpek, Lucia
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