Read by QxMD icon Read


Michael H Rivner, Siyang Liu, Brandy Quarles, Brandi Fleenor, Chengyong Shen, Jinxiu Pan, Lin Mei
INTRODUCTION: The prevalence and characteristics of Agrin and LRP4 antibody positive amyotrophic lateral sclerosis (ALS) patients were studied. METHODS: We tested 82 ALS patients and 59 controls for Agrin and LRP4 antibodies using ELISA. RESULTS: We found that 13.8% of ALS patients had Agrin antibodies, and 9.8% had LRP4 antibodies. Women ALS patients are twice as likely as men to have antibodies. Agrin-positive ALS patients are younger than Agrin-negative ALS patients...
October 18, 2016: Muscle & Nerve
Maddison Turner, Larissa Reid, Mercedes Munkonda, Dylan Burger
OBJECTIVE: Diabetes and hypertension are cognate diseases that often coexist, potentiating one's risk for cardiovascular complications. Both diseases are typified by the development of endothelial dysfunction and are accompanied by increased circulating endothelial microparticles (eMPs). Microparticles are small membrane-derived vesicles that are secreted ubiquitously following cell stress. We have previously shown that eMPs are sensitive markers of vascular injury in hypertension and identified eMP-mediated signaling pathways that lead to endothelial injury...
September 2016: Journal of Hypertension
Daniel B Drachman
The basic abnormality in myasthenia gravis (MG) is a reduction in acetylcholine receptors (AChRs) at neuromuscular junctions due to the effects of autoantibodies that are directed against the AChRs in most patients, or against neighboring proteins involved in the clustering of AChRs (MuSK, LRP-4, or agrin). Clinically, MG is characterized by muscle weakness and fatigue, often in typical patterns. The diagnosis may be missed early, and depends on the recognition of clinical manifestations, the measurement of autoantibodies, and/or electrophysiological features...
October 2016: Seminars in Neurology
B Z Camurdanoglu, C Hrovat, G Dürnberger, M Madalinski, K Mechtler, R Herbst
The neuromuscular junction (NMJ) forms when a motor neuron contacts a muscle fibre. A reciprocal exchange of signals initiates a cascade of signalling events that result in pre- and postsynaptic differentiation. At the centre of these signalling events stands muscle specific kinase (MuSK). MuSK activation, kinase activity and subsequent downstream signalling are crucial for NMJ formation as well as maintenance. Therefore MuSK kinase activity is tightly regulated to ensure proper NMJ development. We have identified a novel serine phosphorylation site at position 751 in MuSK that is increasingly phosphorylated upon agrin stimulation...
September 26, 2016: Scientific Reports
Maddison Turner, Larissa Reid, Mercedes Munkonda, Dylan Burger
OBJECTIVE: Diabetes and hypertension are cognate diseases that often coexist, potentiating one's risk for cardiovascular complications. Both diseases are typified by the development of endothelial dysfunction and are accompanied by increased circulating endothelial microparticles (eMPs). Microparticles are small membrane-derived vesicles that are secreted ubiquitously following cell stress. We have previously shown that eMPs are sensitive markers of vascular injury in hypertension and identified eMP-mediated signaling pathways that lead to endothelial injury...
September 2016: Journal of Hypertension
Dan Yu, Hai-Xia Li, Yi Liu, Ze-Wei Ying, Jing-Jing Guo, Chen-Ying Cao, Jia Wang, Yuan-Fang Li, Hui-Rong Yang
BACKGROUND: C-terminal agrin fragment (CAF) has been shown to be a promising new biomarker for kidney function. The aim of this study was to verify the reference intervals for CAF in Chinese healthy adults and to assess the efficiency of CAF for monitoring renal function after transplantation. METHODS: Serum samples were collected from 200 healthy adult subjects and 60 living donor kidney recipients before and on day 1, day 2 and at 6 months after transplantation...
September 17, 2016: Journal of Clinical Laboratory Analysis
Iris Martinez-Quetglas, Roser Pinyol, Daniel Dauch, Sara Torrecilla, Victoria Tovar, Agrin Moeini, Clara Alsinet, Anna Portela, Leonardo Rodriguez-Carunchio, Manel Solé, Amaia Lujambio, Augusto Villanueva, Swan Thung, Manel Esteller, Lars Zender, Josep M Llovet
BACKGROUND & AIMS: Effective treatments are urgently needed for hepatocellular carcinoma (HCC), which is usually diagnosed at advanced stages. Signaling via the insulin-like growth factor (IGF) pathway is aberrantly activated in HCC by IGF2 overexpression. We aimed to elucidate the mechanism of IGF2 overexpression and its oncogenic activities and evaluate the anti-tumor effects of reducing IGF2 signaling. METHODS: We obtained 228 HCC samples from patients who underwent liver resection, 168 paired non-tumor adjacent cirrhotic liver samples, and 10 non-tumor liver tissues from patients undergoing resection for hepatic hemangioma...
September 7, 2016: Gastroenterology
Dorin-Bogdan Borza
The glomerular basement membrane (GBM) is an essential component of the glomerular filtration barrier. Heparan sulfate proteoglycans such as agrin are major components of the GBM, along with α345(IV) collagen, laminin-521 and nidogen. A loss of GBM heparan sulfate chains is associated with proteinuria in several glomerular diseases and may contribute to the underlying pathology. As the major determinants of the anionic charge of the GBM, heparan sulfate chains have been thought to impart charge selectivity to the glomerular filtration, a view challenged by the negligible albuminuria in mice that lack heparan sulfate in the GBM...
September 6, 2016: Matrix Biology: Journal of the International Society for Matrix Biology
Munetoyo Toda, Morio Ueno, Jun Yamada, Asako Hiraga, Hiroshi Tanaka, Ursula Schlötzer-Schrehardt, Chie Sotozono, Shigeru Kinoshita, Junji Hamuro
PURPOSE: To clarify the adherent properties of cultured human corneal endothelial cell (cHCEC) subpopulations (SPs). METHODS: Each SP was prepared by controlling the culture conditions or by using magnetic cell separation, and then confirmed by staining with several cell-surface markers. Binding abilities of HCEC SPs were examined by adding the cells to culture plates immobilized with collagens, laminins, or proteoglycans, and then centrifuging the plates. Adhered cells were then evaluated by phase-contrast microscopy...
September 1, 2016: Investigative Ophthalmology & Visual Science
Yeong-Jin Choi, Taek Gyoung Kim, Jonghyeon Jeong, Hee-Gyeong Yi, Ji Won Park, Woonbong Hwang, Dong-Woo Cho
Engineered skeletal muscle tissues that mimic the structure and function of native muscle have been considered as an alternative strategy for the treatment of various muscular diseases and injuries. Here, it is demonstrated that 3D cell-printing of decellularized skeletal muscle extracellular matrix (mdECM)-based bioink facilitates the fabrication of functional skeletal muscle constructs. The cellular alignment and the shape of the tissue constructs are controlled by 3D cell-printing technology. mdECM bioink provides the 3D cell-printed muscle constructs with a myogenic environment that supports high viability and contractility as well as myotube formation, differentiation, and maturation...
August 16, 2016: Advanced Healthcare Materials
Jennifer S Myers, Ariana K von Lersner, Qing-Xiang Amy Sang
Protein profiling studies of prostate cancer have been widely used to characterize molecular differences between diseased and non-diseased tissues. When combined with pathway analysis, profiling approaches are able to identify molecular mechanisms of prostate cancer, group patients by cancer subtype, and predict prognosis. This strategy can also be implemented to study prostate cancer in very specific populations, such as African Americans who have higher rates of prostate cancer incidence and mortality than other racial groups in the United States...
2016: Journal of Cancer
Maj Schneider Thomsen, Svend Birkelund, Annette Burkhart, Allan Stensballe, Torben Moos
The brain vascular basement membrane is important for both blood-brain barrier (BBB) development, stability, and barrier integrity and the contribution hereto from brain capillary endothelial cells (BCECs), pericytes, and astrocytes of the BBB is probably significant. The aim of the present study was to analyse four different in vitro models of the murine BBB for expression and possible secretion of major basement membrane proteins from murine BCECs (mBCECs). mBCECs, pericytes, and glial cells (mainly astrocytes and microglia) were prepared from brains of C57BL/6 mice...
July 26, 2016: Journal of Neurochemistry
Bardia Valizadeh, S Agrin Barzanji, Mital Patel, Shakeel Shahdad
This article aims to discuss the use of short dental implants in cleft patients, the construction of an implant-retained obturator and the use of a Createch milled titanium bar with three Locator overdenture attachments incorporated within the bar. CPD/Clinical Relevance: Implant-retained obturators offer a possible solution for treating patients with isolated cleft palate who are struggling with dentures due to the unfavourable soft and hard tissue profile. This paper demonstrates how to manage such patients and shows all the clinical and laboratory stages involved...
April 2016: Dental Update
William D Phillips, Angela Vincent
Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (Ig)G1 and IgG3 antibodies to the acetylcholine receptor (AChR)...
2016: F1000Research
Arezoo Daryadel, Monika Haubitz, Marta Figueiredo, Dominik Steubl, Marcel Roos, Armin Mäder, Stefan Hettwer, Carsten A Wagner
Agrin, a multidomain proteoglycan and neurotrypsin, a neuronal serine protease, are important for forming (neuromuscular) synapses. Proteolytical activity of neurotrypsin produces a C-terminal fragment of agrin, termed CAF, of approximately 22 kDA molecular size which also circulates in blood. The presence of CAF in urine suggests either glomerular filtration or secretion into urine. Blood levels of CAF have been identified as a potential novel marker of kidney function. Here we describe that several nephron segments in the mouse kidney express agrin and neutrotrypsin in addition to the localization of both protein in the glomerulum...
2016: PloS One
Elena Cortés-Vicente, Eduard Gallardo, María Ángeles Martínez, Jordi Díaz-Manera, Luis Querol, Ricard Rojas-García, Isabel Illa
IMPORTANCE: Double-seronegative myasthenia gravis (dSNMG) includes patients with myasthenia gravis (MG) without detectable antibodies to the nicotinic acetylcholine receptor (AChR) or to muscle-specific tyrosine kinase (MuSK). The lack of a biomarker hinders the diagnosis and clinical management in these patients. Cortactin, a protein acting downstream from agrin/low-density lipoprotein receptor-related protein 4 (LRP4)/MuSK, has been described as an antigen in dSNMG. OBJECTIVE: To describe the frequency and clinical features of patients with dSNMG who have cortactin antibodies...
September 1, 2016: JAMA Neurology
Jørgen S Agerholm, Fintan J McEvoy, Fiona Menzi, Vidhya Jagannathan, Cord Drögemüller
BACKGROUND: Bovine arthrogryposis multiplex congenita (AMC) is a syndromic term for a congenital condition characterized by multiple joint contractures. Rare inherited forms of bovine AMC have been reported in different breeds. For AMC in Angus cattle a causative genomic deletion encompassing the agrin (AGRN) gene, encoding an essential neural regulator that induces the aggregation of acetylcholine receptors (AChRs), is known. In 2015, three genetically related cases of generalized AMC affecting Red dairy calves were diagnosed in Denmark...
2016: BMC Genomics
Hiroaki Nakashima, Bisei Ohkawara, Shinsuke Ishigaki, Takayasu Fukudome, Kenyu Ito, Mikito Tsushima, Hiroyuki Konishi, Tatsuya Okuno, Toshiro Yoshimura, Mikako Ito, Akio Masuda, Gen Sobue, Hiroshi Kiyama, Naoki Ishiguro, Kinji Ohno
At the neuromuscular junction (NMJ), acetylcholine receptor (AChR) clustering is mediated by spinal motor neuron (SMN)-derived agrin and its receptors on the muscle, the low-density lipoprotein receptor-related protein 4 (LRP4) and muscle-specific receptor tyrosine kinase (MuSK). Additionally, AChR clustering is mediated by the components of the Wnt pathway. Laser capture microdissection of SMNs revealed that a secreted activator of Wnt signaling, R-spondin 2 (Rspo2), is highly expressed in SMNs. We found that Rspo2 is enriched at the NMJ, and that Rspo2 induces MuSK phosphorylation and AChR clustering...
2016: Scientific Reports
Xiang-Dong Sun, Lei Li, Fang Liu, Zhi-Hui Huang, Jonathan C Bean, Hui-Feng Jiao, Arnab Barik, Seon-Myung Kim, Haitao Wu, Chengyong Shen, Yun Tian, Thiri W Lin, Ryan Bates, Anupama Sathyamurthy, Yong-Jun Chen, Dong-Min Yin, Lei Xiong, Hui-Ping Lin, Jin-Xia Hu, Bao-Ming Li, Tian-Ming Gao, Wen-Cheng Xiong, Lin Mei
Neurotransmission requires precise control of neurotransmitter release from axon terminals. This process is regulated by glial cells; however, the underlying mechanisms are not fully understood. We found that glutamate release in the brain was impaired in mice lacking low-density lipoprotein receptor-related protein 4 (Lrp4), a protein that is critical for neuromuscular junction formation. Electrophysiological studies revealed compromised release probability in astrocyte-specific Lrp4 knockout mice. Lrp4 mutant astrocytes suppressed glutamatergic transmission by enhancing the release of ATP, whose level was elevated in the hippocampus of Lrp4 mutant mice...
August 2016: Nature Neuroscience
Valérie Vilmont, Bruno Cadot, Elsa Vezin, Fabien Le Grand, Edgar R Gomes
The neuromuscular junction (NMJ) allows the transformation of a neuronal message into a mechanical force by muscle contraction and is the target of several neuromuscular disorders. While the neuronal side is under extensive research, the muscle appeared recently to have a growing role in the formation and integrity of the neuromuscular junction. We used an in vitro model of mature myofibers to study the role of dynein on major postsynaptic proteins. We found that dynein affects the expression and the clustering of acetylcholine receptors (AChRs), muscle specific tyrosine kinase (MuSK) and Rapsyn...
2016: Scientific Reports
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"