Arunagiri, Anoop Haataja, Leena Cunningham, Corey N Shrestha, Neha Tsai, Billy Qi, Ling Liu, Ming Arvan, Peter
Published in
Annals of the New York Academy of Sciences
The endoplasmic reticulum (ER) is broadly distributed throughout the cytoplasm of pancreatic beta cells, and this is where all proinsulin is initially made. Healthy beta cells can synthesize 6000 proinsulin molecules per second. Ordinarily, nascent proinsulin entering the ER rapidly folds via the formation of three evolutionarily conserved disulfid...
Cron, Mélanie A Maillard, Solène Villegas, José Truffault, Frédérique Sudres, Muriel Dragin, Nadine Berrih-Aknin, Sonia Le Panse, Rozen
Published in
Annals of the New York Academy of Sciences
It has long been established that the thymus plays a central role in autoimmune myasthenia gravis (MG) because of either thymoma or thymic hyperplasia of lymphoproliferative origin. In this review, we discuss thymic changes associated with thymic hyperplasia and their implications in the development of an autoimmune response against the acetylcholi...
Meriney, Stephen D Tarr, Tyler B Ojala, Kristine S Wu, Man Li, Yizhi Lacomis, David Garcia-Ocaña, Adolfo Liang, Mary Valdomir, Guillermo Wipf, Peter
...
Published in
Annals of the New York Academy of Sciences
Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder caused by antibodies directed against the voltage-gated calcium channels that provide the calcium ion flux that triggers acetylcholine release at the neuromuscular junction. To study the pathophysiology of LEMS and test candidate therapeutic strategies, a passive-transfer animal mod...
Ghazanfari, Nazanin Trajanovska, Sofie Morsch, Marco Liang, Simon X Reddel, Stephen W Phillips, William D
Published in
Annals of the New York Academy of Sciences
While the majority of myasthenia gravis patients express antibodies targeting the acetylcholine receptor, the second most common cohort instead displays autoantibodies against muscle-specific kinase (MuSK). MuSK is a transmembrane tyrosine kinase found in the postsynaptic membrane of the neuromuscular junction. During development, MuSK serves as a ...
Punga, Anna Rostedt Punga, Tanel
Published in
Annals of the New York Academy of Sciences
MicroRNAs (miRNAs) are small noncoding RNA molecules that bind to specific mRNA targets and regulate a wide range of important biological processes within cells. Circulating miRNAs are released into the extracellular space and can be measured in most biofluids, including blood serum and plasma. Recently, circulating miRNAs have emerged as easily ac...
Devarakonda, Kavya Stanley, Sarah
Published in
Annals of the New York Academy of Sciences
The central nervous system (CNS) plays a vital role in regulating energy balance and metabolism. Over the last 50 years, studies in animal models have allowed us to identify critical CNS regions involved in these processes and even crucial cell populations. Now, techniques for genetically and anatomically targeted manipulation of specific neural po...
Sussman, Jon Farrugia, Maria E Maddison, Paul Hill, Marguerite Leite, M Isabel Hilton-Jones, David
Published in
Annals of the New York Academy of Sciences
Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction for which many therapies were developed before the era of evidence-based medicine. Despite the basic principles of treatment being well known, patients continue to receive suboptimal treatment. A myasthenia gravis guidelines group was therefore established under the aegis...
Knezevic, Ivana Griffiths, Elwyn
Published in
Annals of the New York Academy of Sciences
The most advanced regulatory processes for complex biological products have been put in place in many countries to provide appropriate regulatory oversight of biotherapeutic products in general, and similar biotherapeutics in particular. This process is still ongoing and requires regular updates to national regulatory requirements in line with scie...
Sauer, Reine-Solange Kirchner, Juliane Yang, Shaobing Hu, Liu Leinders, Mathias Sommer, Claudia Brack, Alexander Rittner, Heike L
Published in
Annals of the New York Academy of Sciences
The blood-spinal cord barrier (BSCB) prevents leakage of molecules, such as pronociceptive mediators, into the spinal cord, but its role in the pathophysiology of neuropathic pain is not completely understood. Rats with chronic constriction injury (CCI) develop mechanical allodynia, thermal hypersensitivity, and reduced motor performance (Rota-Rod ...
Schuetz, Anja Radusheva, Veselina Krug, Susanne M Heinemann, Udo
Published in
Annals of the New York Academy of Sciences
Tricellulin is a tight junction protein localized to tricellular contacts in many epithelial tissues, where it is required for full barrier control. Here, we present crystal structures of the tricellulin C-terminal coiled-coil domain, revealing a potential dimeric arrangement. By combining structural, biochemical, functional, and mutation analyses,...
