Faculty Opinions recommendation of Antibodies against muscle-specific kinase impair both presynaptic and postsynaptic functions in a murine model of myasthenia gravis.

Author(s):  
Angela Vincent
2012 ◽  
Vol 180 (2) ◽  
pp. 798-810 ◽  
Author(s):  
Shuuichi Mori ◽  
Sachiho Kubo ◽  
Takuyu Akiyoshi ◽  
Shigeru Yamada ◽  
Tsuyoshi Miyazaki ◽  
...  

2014 ◽  
Vol 24 (2) ◽  
pp. 148-150 ◽  
Author(s):  
Zeinab Heidarzadeh ◽  
Seyyed-Asadollah Mousavi ◽  
Vahid Reza Ostovan ◽  
Shahriar Nafissi

2018 ◽  
Vol 1413 (1) ◽  
pp. 111-118 ◽  
Author(s):  
Jan J.G.M. Verschuuren ◽  
Jaap J. Plomp ◽  
Steve J. Burden ◽  
Wei Zhang ◽  
Yvonne E. Fillié-Grijpma ◽  
...  

2017 ◽  
Vol 14 (1) ◽  
pp. 14-17
Author(s):  
Rajeev Ojha ◽  
Krishna K Oli ◽  
Bikram P Gajurel ◽  
Ragesh Karn ◽  
Reema Rajbhandari ◽  
...  

Myasthenia gravis (MG) has a cosmopolitan distribution and can affect people of all age group. Sometimes atypical presentation causes difficulty in the early diagnosis and management. Our aim is to study the clinical subtypes and manifestations of MG along with serological and electrophysiological diagnostic methods. Patients who were admitted in Neurology department or presented in Neurology outpatient department of Tribhuvan University Teaching Hospital from 2015 March to 2016 November were retrospectively reviewed. Out of 28 patients reviewed, 23 patients were included in the study. Their mean age of onset was 40.4±19.2 years; range=12 – 78 years, and 12 of them were female (52.2%). Eight patients (34.8%) were diagnosed with ocular myasthenia and 15 were patients (65.2%) of General Myasthenia. Seventeen patients (73.9%) were acetylcholine receptor (AChR) antibodies positive, one female patient was found to be muscle specific kinase (MuSK) positive. Decremental pattern in Repetitive Nerve stimulation (RNS) was reported in 11 patients (47.8%) and ice pack test was positive in 16 patients (69.3%). Ophthalmological findings are the most common presentation of MG patients. Icepack test is an easy clinical diagnostic tool for outpatient department which has both high sensitivity and specificity. RNS and antibody tests are the supporting tests useful for confirming the diagnosis.Nepal Journal of Neuroscience, Volume 14, Number 1, 2017, Page: 14-17


2020 ◽  
Vol 7 (1) ◽  
pp. 47-50
Author(s):  
T.J.S. Bekooij ◽  
H.J. Gilhuis ◽  
L. Dawson ◽  
E.H. Niks

2019 ◽  
Vol 8 ◽  
pp. 184945441987591 ◽  
Author(s):  
Antonio Junior Lepedda ◽  
Giovanni Andrea Deiana ◽  
Omar Lobina ◽  
Gabriele Nieddu ◽  
Paola Baldinu ◽  
...  

Myasthenia gravis (MG) is an autoimmune disease leading to varying degrees of skeletal muscle weakness. It is caused by specific antibodies directed against definite components in the postsynaptic membrane at the neuromuscular junction (NMJ), such as the acetylcholine receptor (AChR) and the muscle-specific kinase (MUSK) receptor. In clinical practice, MG patients may be classified into three main subgroups based on the occurrence of serum autoantibodies directed against AChR or MUSK receptor or antibody-negative. As the MG subgroups differ in terms of clinical characteristics, disease pathogenesis, prognosis, and response to therapies, they could benefit from targeted treatment as well as the detection of other possible disease biomarkers. We performed proteomics on plasma fractions enriched in low-abundance proteins to identify potential biomarkers according to different autoimmune responses. By this approach, we evidenced a significant reduction of vitronectin in MG patients compared to healthy controls, irrespective of the autoantibodies NMJ target. The obtained results were validated by mono- and two-dimensional Western blotting analysis. Vitronectin is a multifunctional glycoprotein involved in the regulation of several pathophysiological processes, including complement-dependent immune response, coagulation, fibrinolysis, pericellular proteolysis, cell attachment, and spreading. The pathophysiological significance of the reduction of plasma vitronectin in MG patients has yet to be fully elucidated. It could be related either to a possible deposition of vitronectin at NMJ to counteract the complement-mediated muscle damage at this level or to a parallel variation of this glycoprotein in the muscle extracellular matrix with secondary induced alteration in clustering of AChRs at NMJ, as it occurs with variation in concentrations of agrin, another extracellular matrix component. The clinical value of measuring plasma vitronectin has yet to be defined. According to present findings, significantly lower plasma values of this glycoprotein might be indicative of an impaired complement-dependent immune response.


2019 ◽  
Vol 60 (5) ◽  
Author(s):  
Joome Suh ◽  
Benjamin R. Slawski ◽  
Aidan A. Long ◽  
Amanda C. Guidon

2015 ◽  
Vol 2 (2) ◽  
pp. e77 ◽  
Author(s):  
Jeffrey T. Guptill ◽  
John S. Yi ◽  
Donald B. Sanders ◽  
Amanda C. Guidon ◽  
Vern C. Juel ◽  
...  

2016 ◽  
Vol 26 (6) ◽  
pp. 350-353 ◽  
Author(s):  
Maartje G. Huijbers ◽  
Erik H. Niks ◽  
Rinse Klooster ◽  
Marianne de Visser ◽  
Jan B. Kuks ◽  
...  

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