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https://www.readbyqxmd.com/read/28626780/igg-specific-cell-based-assay-detects-potentially-pathogenic-musk-abs-in-seronegative-mg
#1
Saif Huda, Patrick Waters, Mark Woodhall, Maria Isabel Leite, Leslie Jacobson, Anna De Rosa, Michelangelo Maestri, Roberta Ricciardi, Jeannine M Heckmann, Angelina Maniaol, Amelia Evoli, Judy Cossins, David Hilton-Jones, Angela Vincent
OBJECTIVE: To increase the detection of MuSK-Abs using a CBA and test their pathogenicity. METHODS: Sera from 69 MuSK-RIA-positive patients with myasthenia gravis (MG) (Definite MuSK-MG), 169 patients negative for MuSK-RIA and AChR-RIA (seronegative MG, SNMG), 35 healthy individuals (healthy controls, HCs), and 16 NMDA receptor-Ab-positive (NMDAR-Ab) disease controls were tested for binding to MuSK on a CBA using different secondary antibodies. RESULTS: Initially, in addition to 18% of SNMG sera, 11% of HC and 19% of NMDAR-Ab sera showed positive binding to MuSK-transfected cells; this low specificity was due to anti-IgG(H+L) detection of IgM bound nonspecifically to MuSK...
July 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28581497/the-extracellular-matrix-protein-agrin-promotes-heart-regeneration-in-mice
#2
Elad Bassat, Yara Eid Mutlak, Alex Genzelinakh, Ilya Y Shadrin, Kfir Baruch-Umansky, Oren Yifa, David Kain, Dana Rajchman, John Leach, Daria Riabov Bassat, Yael Udi, Rachel Sarig, Irit Sagi, James F Martin, Nenad Bursac, Shenhav Cohen, Eldad Tzahor
The adult mammalian heart is non-regenerative due to the post-mitotic nature of cardiomyocytes. The neonatal mouse heart can regenerate, but only for the first week of life. Here we show that changes in the composition of the extracellular matrix (ECM) during this week can affect cardiomyocyte growth and differentiation in mice. We identify Agrin, a component of neonatal ECM, as required for the full regenerative capacity of neonatal mouse hearts. In vitro, recombinant Agrin promotes the division of mouse and human iPSC-derived cardiomyocytes via a mechanism that involves the disassembly of the dystrophin glycoprotein complex and Yap and ERK-mediated signaling...
June 5, 2017: Nature
https://www.readbyqxmd.com/read/28555889/reduced-muscle-strength-in-ether-lipid-deficient-mice-is-accompanied-by-altered-development-and-function-of-the-neuromuscular-junction
#3
Fabian Dorninger, Ruth Herbst, Bojana Kravic, Bahar Z Camurdanoglu, Igor Macinkovic, Gerhard Zeitler, Sonja Forss-Petter, Siegfried Strack, Muzamil Majid Khan, Hans R Waterham, Rüdiger Rudolf, Said Hashemolhosseini, Johannes Berger
Inherited deficiency in ether lipids, a subgroup of phospholipids whose biosynthesis needs peroxisomes, causes the fatal human disorder rhizomelic chondrodysplasia punctata. The exact roles of ether lipids in the mammalian organism and, therefore, the molecular mechanisms underlying the disease are still largely enigmatic. Here, we used the glyceronephosphate O-acyltransferase knockout (Gnpat KO) mouse to study the consequences of complete inactivation of ether lipid biosynthesis and documented substantial deficits in motor performance and muscle strength of these mice...
May 29, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28516329/screening-for-lipoprotein-receptor-related-protein-4-agrin-and-titin-antibodies-and-exploring-the-autoimmune-spectrum-in-myasthenia-gravis
#4
Isabell Cordts, Nicolas Bodart, Kathi Hartmann, Katerina Karagiorgou, John S Tzartos, Lin Mei, Jens Reimann, Philip Van Damme, Michael H Rivner, Alain Vigneron, Joachim Weis, Jörg B Schulz, Socrates J Tzartos, Kristl G Claeys
In autoimmune myasthenia gravis (MG), the identification of antibodies and characterization of serological subgroups is of great importance for diagnosis and management of the disease. Our aims were to study the frequency of antibodies against lipoprotein-related protein 4 (LRP4), agrin, and titin using the most recent techniques, and to characterize corresponding clinical features and autoimmune diseases (AID) in 100 MG-patients. The antibody frequencies in the 55 AChR-antibody positive patients were 7% LRP4, 5% agrin, 53% titin, and in the 45 AChR-antibody negative patients 2% MuSK, 2% LRP4, 2% agrin, and 27% titin...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28493069/comparison-of-molecular-marker-expression-in-early-zebrafish-brain-development-following-chronic-ethanol-or-morpholino-treatment
#5
Chengjin Zhang, Oswald Boa-Amponsem, Gregory J Cole
This study was undertaken to ascertain whether defined markers of early zebrafish brain development are affected by chronic ethanol exposure or morpholino knockdown of agrin, sonic hedgehog, retinoic acid, and fibroblast growth factors, four signaling molecules that are suggested to be ethanol sensitive. Zebrafish embryos were exposed to 2% ethanol from 6 to 24 hpf or injected with agrin, shha, aldh1a3, or fgf8a morpholinos. In situ hybridization was employed to analyze otx2, pax6a, epha4a, krx20, pax2a, fgf8a, wnt1, and eng2b expression during early brain development...
May 10, 2017: Experimental Brain Research. Experimentelle Hirnforschung. Expérimentation Cérébrale
https://www.readbyqxmd.com/read/28441759/synaptic-homeostasis-and-its-immunological-disturbance-in-neuromuscular-junction-disorders
#6
REVIEW
Masaharu Takamori
In the neuromuscular junction, postsynaptic nicotinic acetylcholine receptor (nAChR) clustering, trans-synaptic communication and synaptic stabilization are modulated by the molecular mechanisms underlying synaptic plasticity. The synaptic functions are based presynaptically on the active zone architecture, synaptic vesicle proteins, Ca(2+) channels and synaptic vesicle recycling. Postsynaptically, they are based on rapsyn-anchored nAChR clusters, localized sensitivity to ACh, and synaptic stabilization via linkage to the extracellular matrix so as to be precisely opposed to the nerve terminal...
April 24, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28441579/geldanamycin-inspired-compounds-induce-direct-trans-differentiation-of-human-mesenchymal-stem-cells-to-neurons
#7
Srinivas Jogula, Anand Ram Soorneedi, Jagan Gaddam, Srinivas Chamakuri, Girdhar Singh Deora, Ranjith Kumar Indarapu, Murali Krishna Ramgopal, Subhadra Dravida, Prabhat Arya
Inspired from geldanamycin, the synthesis of a new series of 20-membered macrocyclic compounds is developed. The key features in our design are (i) retention of the fragment having the precise chiral functional groups of geldanamycin at C10, C11, C12 and C14, and (ii) replacement of an olefin moiety with the ester group, and the quinoid sub-structure with the triazole ring. The southern fragment needed for the macrocyclic ring formation was obtained from Evans' syn aldol as the key reaction and with the use of D-mannitol as the cheap source of a chiral starting material...
April 18, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28379354/motor-neuronal-repletion-of-the-nmj-organizer-agrin-modulates-the-severity-of-the-spinal-muscular-atrophy-disease-phenotype-in-model-mice
#8
Jeong-Ki Kim, Charlotte Caine, Tomoyuki Awano, Ruth Herbst, Umrao R Monani
Spinal muscular atrophy (SMA) is a common and often fatal neuromuscular disorder caused by low levels of the Survival Motor Neuron (SMN) protein. Amongst the earliest detectable consequences of SMN deficiency are profound defects of the neuromuscular junctions (NMJs). In model mice these synapses appear disorganized, fail to mature and are characterized by poorly arborized nerve terminals. Given one role of the SMN protein in orchestrating the assembly of spliceosomal snRNP particles and subsequently regulating the alternative splicing of pre-mRNAs, a plausible link between SMN function and the distal neuromuscular SMA phenotype is an incorrectly spliced transcript or transcripts involved in establishing or maintaining NMJ structure...
July 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28357490/annexin-a10-optimally-differentiates-between-intrahepatic-cholangiocarcinoma-and-hepatic-metastases-of-pancreatic-ductal-adenocarcinoma-a-comparative-study-of-immunohistochemical-markers-and-panels
#9
Julia Kälsch, Juliet Padden, Stefanie Bertram, Leona L Pott, Henning Reis, Daniela Westerwick, Christoph M Schaefer, Jan-P Sowa, Dorothe Möllmann, Christian Fingas, Alexander Dechȇne, Barbara Sitek, Martin Eisenacher, Ali Canbay, Maike Ahrens, Hideo A Baba
Discriminating intrahepatic cholangiocarcinoma (ICC) from hepatic metastases of pancreatic ductal adenocarcinoma (mPDAC) can be challenging. While pathologists might depend on clinical information regarding a primary tumor, their diagnosis will lead the patient either to potentially curative surgery (for ICC) or to palliation (for mPDAC). Beyond the validation of recently published potential biomarkers for PDAC (primary or metastatic) in a large cohort, we assessed diagnostic performance of the most promising candidates in the challenging task of discriminating metastatic PDAC (mPDAC) from ICC...
May 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28324731/basement-membranes
#10
Ranjay Jayadev, David R Sherwood
Basement membranes (BMs) are thin, dense sheets of specialized, self-assembled extracellular matrix that surround most animal tissues (Figure 1, top). The emergence of BMs coincided with the origin of multicellularity in animals, suggesting that they were essential for the formation of tissues. Their sheet-like structure derives from two independent polymeric networks - one of laminin and one of type IV collagen (Figure 1, bottom). These independent collagen and laminin networks are thought to be linked by several additional extracellular matrix proteins, including nidogen and perlecan (Figure 1, bottom)...
March 20, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28298885/functional-roles-of-the-interaction-of-app-and-lipoprotein-receptors
#11
REVIEW
Theresa Pohlkamp, Catherine R Wasser, Joachim Herz
The biological fates of the key initiator of Alzheimer's disease (AD), the amyloid precursor protein (APP), and a family of lipoprotein receptors, the low-density lipoprotein (LDL) receptor-related proteins (LRPs) and their molecular roles in the neurodegenerative disease process are inseparably interwoven. Not only does APP bind tightly to the extracellular domains (ECDs) of several members of the LRP group, their intracellular portions are also connected through scaffolds like the one established by FE65 proteins and through interactions with adaptor proteins such as X11/Mint and Dab1...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28273460/agrin-as-a-mechanotransduction-signal-regulating-yap-through-the-hippo-pathway
#12
Sayan Chakraborty, Kizito Njah, Ajaybabu V Pobbati, Ying Bena Lim, Anandhkumar Raju, Manikandan Lakshmanan, Vinay Tergaonkar, Chwee Teck Lim, Wanjin Hong
The Hippo pathway effectors YAP and TAZ act as nuclear sensors of mechanical signals in response to extracellular matrix (ECM) cues. However, the identity and nature of regulators in the ECM and the precise pathways relaying mechanoresponsive signals into intracellular sensors remain unclear. Here, we uncover a functional link between the ECM proteoglycan Agrin and the transcriptional co-activator YAP. Importantly, Agrin transduces matrix and cellular rigidity signals that enhance stability and mechanoactivity of YAP through the integrin-focal adhesion- and Lrp4/MuSK receptor-mediated signaling pathways...
March 7, 2017: Cell Reports
https://www.readbyqxmd.com/read/28228399/accumulation-of-worn-out-gbm-material-substantially-contributes-to-mesangial-matrix-expansion-in-diabetic-nephropathy
#13
Wilhelm Kriz, Jana Löwen, Giuseppina Federico, Jacob van den Born, Elisabeth Gröne, Hermann-Josef Gröne
Thickening of the glomerular basement membrane (GBM) and expansion of the mesangial matrix are hallmarks of diabetic nephropathy (DN) generally considered to emerge from different sites of overproduction; GBM components from podocytes and mesangial matrix from mesangial cells. Re-evaluation of 918 biopsies with DN revealed strong evidence that these mechanisms are connected to each other, wherein excess GBM components fail to undergo degradation and are deposited in the mesangium. These data do not exclude that also mesangial cells synthesize components that contribute to the accumulation of matrix in the mesangium...
February 22, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28221305/a-novel-missense-variant-in-the-agrn-gene-congenital-myasthenic-syndrome-presenting-with-head-drop
#14
Mert Karakaya, Ozge Ceyhan-Birsoy, Alan H Beggs, Haluk Topaloglu
Congenital myasthenic syndromes (CMS) are a heterogeneous group of diseases of the neuromuscular junction caused by compromised synaptic transmission. Clinical features include early-onset weakness of limbs and oculobulbar muscles resulting in hypotonia, bulbar paresis, ptosis, and hypoventilation. The first dropped head syndrome in children were detected in 2 patients with LMNA and SEPN1 mutations. We report a 17-month-old boy with dropped head and limb-girdle weakness, who had no ptosis or ophthalmoplegia at presentation...
March 2017: Journal of Clinical Neuromuscular Disease
https://www.readbyqxmd.com/read/28127382/dietary-supplementation-with-bovine-derived-milk-fat-globule-membrane-lipids-promotes-neuromuscular-development-in-growing-rats
#15
James F Markworth, Brenan Durainayagam, Vandre C Figueiredo, Karen Liu, Jian Guan, Alastair K H MacGibbon, Bertram Y Fong, Aaron C Fanning, Angela Rowan, Paul McJarrow, David Cameron-Smith
BACKGROUND: The milk fat globule membrane (MFGM) is primarily composed of polar phospho- and sphingolipids, which have established biological effects on neuroplasticity. The present study aimed to investigate the effect of dietary MFGM supplementation on the neuromuscular system during post-natal development. METHODS: Growing rats received dietary supplementation with bovine-derived MFGM mixtures consisting of complex milk lipids (CML), beta serum concentrate (BSC) or a complex milk lipid concentrate (CMLc) (which lacks MFGM proteins) from post-natal day 10 to day 70...
2017: Nutrition & Metabolism
https://www.readbyqxmd.com/read/28126467/mixed-hepatocellular-cholangiocarcinoma-tumors-cholangiolocellular-carcinoma-is-a-distinct-molecular-entity
#16
Agrin Moeini, Daniela Sia, Zhongyang Zhang, Genis Camprecios, Ashley Stueck, Hui Dong, Robert Montal, Laura Torrens, Iris Martinez-Quetglas, M Isabel Fiel, Ke Hao, Augusto Villanueva, Swan N Thung, Myron E Schwartz, Josep M Llovet
BACKGROUND & AIMS: Mixed hepatocellular cholangiocarcinoma (HCC-CCA) is a rare and poorly understood type of primary liver cancer. We aimed to perform a comprehensive molecular characterization of this malignancy. METHODS: Gene expression profiling, DNA copy number detection, and exome sequencing using formalin-fixed samples from 18 patients with mixed HCC-CCA were performed, encompassing the whole histological spectrum of the disease. Comparative genomic analysis was carried out, using independent datasets of HCC (n=164) and intrahepatic cholangiocarcinoma (iCCA) (n=149)...
January 23, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28123487/brain-derived-and-glial-cell-line-derived-neurotrophic-factor-fusion-protein-immobilization-to-laminin
#17
Baoxin Wang, Junjie Yuan, Jiafeng Xu, Xinwei Chen, Xinjiang Ying, Pin Dong
Damage to the recurrent laryngeal nerve often causes hoarseness, dyspnea, dysphagia, and sometimes asphyxia due to vocal cord paralysis which result in a reduction of quality of life. Brain-derived neurotrophic factor (BDNF) and glial cell line-derived neurotrophic factor (GDNF) play critical roles in peripheral nerve regeneration. However, methods for efficiently delivering these molecules are lacking, which limits their use in clinical applications. The present study reports an effective strategy for targeting BDNF and GDNF to laminin by fusing the N-terminal domains of these molecules with agrin (NtA)...
January 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28107841/adeno-associated-virus-mediated-mini-agrin-delivery-is-unable-to-rescue-disease-phenotype-in-a-mouse-model-of-limb-girdle-muscular-dystrophy-type-2i
#18
Charles H Vannoy, Haowen Zhou, Chunping Qiao, Xiao Xiao, Anne G Bang, Qi L Lu
Agrin is a basement membrane-specific proteoglycan that can regulate orientation of cytoskeleton proteins and improve function of dystrophic skeletal muscle. In skeletal muscle, agrin binds with high affinity to laminin(s) and α-dystroglycan (α-DG), an integral part of the dystrophin-glycoprotein complex. Miniaturized forms of agrin (mAgrin) have been shown to ameliorate disease pathology in a laminin-α2 knockout mouse model of muscular dystrophy, acting as a link between α-DG and laminin(s). Here, we test whether mAgrin might also improve pathologies associated with FKRP-related dystroglycanopathies, another form of muscular dystrophy characterized by weak interactions between muscle and basement membranes...
February 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28072465/splicing-regulation-and-dysregulation-of-cholinergic-genes-expressed-at-the-neuromuscular-junction
#19
REVIEW
Kinji Ohno, Mohammad Alinoor Rahman, Mohammad Nazim, Farhana Nasrin, Yingni Lin, Jun-Ichi Takeda, Akio Masuda
We humans have evolved by acquiring diversity of alternative RNA metabolisms including alternative means of splicing and transcribing non-coding genes, and not by acquiring new coding genes. Tissue-specific and developmental stage-specific alternative RNA splicing is achieved by tightly regulated spatiotemporal regulation of expressions and activations of RNA-binding proteins that recognize their cognate splicing cis-elements on nascent RNA transcripts. Genes expressed at the neuromuscular junction (NMJ) are also alternatively spliced...
January 10, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28069562/effect-of-electroacupuncture-on-the-expression-of-agrin-and-acetylcholine-receptor-subtypes-in-rats-with-tibialis-anterior-muscular-atrophy-induced-by-sciatic-nerve-injection-injury
#20
Jianqi Yu, Meng Wang, Junying Liu, Xiaoming Zhang, Shengbo Yang
OBJECTIVE: To investigate the effects of electroacupuncture (EA) on mRNA and protein expression of agrin, acetylcholine receptor (AChR)-ε and AChR-γ in a rat model of tibialis anterior muscle atrophy induced by sciatic nerve injection injury, and to examine the underlying mechanism of action. METHODS: Fifty-four adult Sprague-Dawley rats were divided into four groups: healthy control group (CON, n=6); sciatic nerve injury group (SNI, n=24), comprising rats euthanased at 1, 2, 4 and 6 weeks, respectively, after penicillin injection-induced SNI (n=6 each); CON+EA group (n=12), comprising healthy rats euthanased at 4 and 6 weeks (after 2 and 4 weeks, respectively, of EA at GB30 and ST36); and SNI+EA group, comprising rats euthanased at 4 and 6 weeks (after 2 and 4 weeks, respectively, of EA)...
January 9, 2017: Acupuncture in Medicine: Journal of the British Medical Acupuncture Society
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