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https://www.readbyqxmd.com/read/27920424/ultrastructural-characterization-of-the-lower-motor-system-in-a-mouse-model-of-krabbe-disease
#1
Valentina Cappello, Laura Marchetti, Paola Parlanti, Silvia Landi, Ilaria Tonazzini, Marco Cecchini, Vincenzo Piazza, Mauro Gemmi
Krabbe disease (KD) is a neurodegenerative disorder caused by the lack of β- galactosylceramidase enzymatic activity and by widespread accumulation of the cytotoxic galactosyl-sphingosine in neuronal, myelinating and endothelial cells. Despite the wide use of Twitcher mice as experimental model for KD, the ultrastructure of this model is partial and mainly addressing peripheral nerves. More details are requested to elucidate the basis of the motor defects, which are the first to appear during KD onset. Here we use transmission electron microscopy (TEM) to focus on the alterations produced by KD in the lower motor system at postnatal day 15 (P15), a nearly asymptomatic stage, and in the juvenile P30 mouse...
December 2016: Scientific Reports
https://www.readbyqxmd.com/read/27893494/a-comparison-of-magnetic-resonance-imaging-and-electroneuromyography-for-denervated-muscle-diagnosis
#2
Betül Tepeli, Metin Karataş, Mehmet Coşkun, Oya Ümit Yemişçi
INTRODUCTION: The aim of this study was to evaluate the diagnostic value of magnetic resonance imaging (MRI) for muscle denervation due to focal lesions of the median nerve and to compare electrophysiological findings with MRI findings. METHODS: Twenty-six patients with electrophysiological studies diagnose for focal lesions of the median nerve were included in this study. Electrophysiological studies and MRI were conducted on 34 patients' hands. Patient hands were divided into two groups based on edema findings revealed by the MRI: group 1 (edema-negative group; n = 24) and group 2 (edema-positive group; n = 10)...
November 21, 2016: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/27891474/an-evaluation-of-use-of-botulinum-toxin-type-a-in-the-management-of-dynamic-forehead-wrinkles-a-clinical-study
#3
Avvaru Susmita, Naga Neelima Devi Kolli, Sridhar Meka, Srinivas Pandi Chakravarthi, Vivekanand Sabanna Kattimani, Krishna Prasad Lingamaneni, Latheef Saheb Shaik
INTRODUCTION: The pursuit of youth and beauty has undergone a resurgence of interest which is evidenced by increasing cosmetic procedures. Botulinum Toxin Type A (Botox) is one among the many procedures invented for facial rejuvenation which denervates certain muscles of facial expression responsible for facial wrinkles. It has been applied in the forehead, glabella, lateral canthal area and neck. In maxillofacial area hyperactive forehead wrinkles show sagging. AIM: This study was aimed to clinically evaluate the efficacy of Botox injection in the elimination of hyperdynamic forehead wrinkles and the objectives were to compare pre-operative and post-operative improvement in the number of wrinkles, photographic grading and patient satisfaction responses after 1(st) week, 4(th) week and 16(th) week...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27875560/denervation-induced-activation-of-the-standard-proteasome-and-immunoproteasome
#4
Haiming M Liu, Deborah A Ferrington, Cory W Baumann, LaDora V Thompson
The standard 26S proteasome is responsible for the majority of myofibrillar protein degradation leading to muscle atrophy. The immunoproteasome is an inducible form of the proteasome. While its function has been linked to conditions of atrophy, its contribution to muscle proteolysis remains unclear. Therefore, the purpose of this study was to determine if the immunoproteasome plays a role in skeletal muscle atrophy induced by denervation. Adult male C57BL/6 wild type (WT) and immunoproteasome knockout lmp7-/-/mecl-1-/- (L7M1) mice underwent tibial nerve transection on the left hindlimb for either 7 or 14 days, while control mice did not undergo surgery...
2016: PloS One
https://www.readbyqxmd.com/read/27836905/electrophysiological-study-of-femoral-nerve-function-after-a-continuous-femoral-nerve-block-for-anterior-cruciate-ligament-reconstruction-a-randomized-controlled-single-blind-trial
#5
Kevin Stebler, Robin Martin, Kyle Robert Kirkham, Thierry Küntzer, Istvan Bathory, Eric Albrecht
BACKGROUND: A continuous femoral nerve block (CFNB) is an effective analgesic treatment after anterior cruciate ligament (ACL) reconstruction but may result in transient femoral nerve injuries and quadriceps muscle weakness, which in turn contribute to worsened functional outcomes. PURPOSE: To compare electrophysiological criteria of a femoral nerve injury as well as functional and pain-related outcomes after ACL reconstruction when analgesia was provided by a CFNB or intravenous patient-controlled analgesic of morphine (IV PCA)...
November 11, 2016: American Journal of Sports Medicine
https://www.readbyqxmd.com/read/27834214/motor-neuron-disease-tdp-43-pathology-and-memory-deficits-in-mice-expressing-als-ftd-linked-ubqln2-mutations
#6
Nhat T T Le, Lydia Chang, Irina Kovlyagina, Polymnia Georgiou, Nathaniel Safren, Kerstin E Braunstein, Mark D Kvarta, Adam M Van Dyke, Tara A LeGates, Thomas Philips, Brett M Morrison, Scott M Thompson, Adam C Puche, Todd D Gould, Jeffrey D Rothstein, Philip C Wong, Mervyn J Monteiro
Missense mutations in ubiquilin 2 (UBQLN2) cause ALS with frontotemporal dementia (ALS-FTD). Animal models of ALS are useful for understanding the mechanisms of pathogenesis and for preclinical investigations. However, previous rodent models carrying UBQLN2 mutations failed to manifest any sign of motor neuron disease. Here, we show that lines of mice expressing either the ALS-FTD-linked P497S or P506T UBQLN2 mutations have cognitive deficits, shortened lifespans, and develop motor neuron disease, mimicking the human disease...
November 22, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27826939/a-novel-iron-chelator-radical-scavenger-ameliorates-motor-dysfunction-and-improves-life-span-and-mitochondrial-biogenesis-in-sod1-g93a-als-mice
#7
Sagit Golko-Perez, Tamar Amit, Orit Bar-Am, Moussa B H Youdim, Orly Weinreb
The aim of the present study was to evaluate the therapeutic effect of the novel neuroprotective multitarget brain permeable monoamine oxidase inhibitor/iron chelating-radical scavenging drug, VAR10303 (VAR), co-administered with high-calorie/energy-supplemented diet (ced) in SOD1(G93A) transgenic amyotrophic lateral sclerosis (ALS) mice. Administration of VAR-ced was initiated after the appearance of disease symptoms (at day 88), as this regimen is comparable with the earliest time at which drug therapy could start in ALS patients...
November 8, 2016: Neurotoxicity Research
https://www.readbyqxmd.com/read/27813490/insights-into-the-role-and-regulation-of-tctp-in-skeletal-muscle
#8
Craig A Goodman, Allison M Coenen, John W Frey, Jae-Sung You, Robert G Barker, Barnaby P Frankish, Robyn M Murphy, Troy A Hornberger
The translationally controlled tumor protein (TCTP) is upregulated in a range of cancer cell types, in part, by the activation of the mechanistic target of rapamycin (mTOR). Recently, TCTP has also been proposed to act as an indirect activator of mTOR. While it is known that mTOR plays a major role in the regulation of skeletal muscle mass, very little is known about the role and regulation of TCTP in this post-mitotic tissue. This study shows that muscle TCTP and mTOR signaling are upregulated in a range of mouse models (mdx mouse, mechanical load-induced hypertrophy, and denervation- and immobilization-induced atrophy)...
November 1, 2016: Oncotarget
https://www.readbyqxmd.com/read/27796491/nerve-supply-of-the-subscapularis-during-anterior-shoulder-surgery-definition-of-a-potential-risk-area
#9
Tim Leschinger, Michael Hackl, Felix Zeifang, Martin Scaal, Lars Peter Müller, Kilian Wegmann
PURPOSE: The purpose of the study was to evaluate the position of the subscapular nerves relative to surgical landmarks during exposure and to analyze the pattern of innervation of the subscapularis to avoid injury during anterior shoulder surgery. METHODS: 20 embalmed human cadaveric shoulder specimens were used in the study. The muscular insertions of the subscapular nerves were marked and their closest branches to the musculotendinous junction and the coracoid process were measured in horizontal and vertical distances...
October 28, 2016: Archives of Orthopaedic and Trauma Surgery
https://www.readbyqxmd.com/read/27796305/monitoring-peripheral-nerve-degeneration-in-als-by-label-free-stimulated-raman-scattering-imaging
#10
Feng Tian, Wenlong Yang, Daniel A Mordes, Jin-Yuan Wang, Johnny S Salameh, Joanie Mok, Jeannie Chew, Aarti Sharma, Ester Leno-Duran, Satomi Suzuki-Uematsu, Naoki Suzuki, Steve S Han, Fa-Ke Lu, Minbiao Ji, Rosanna Zhang, Yue Liu, Jack Strominger, Neil A Shneider, Leonard Petrucelli, X Sunney Xie, Kevin Eggan
The study of amyotrophic lateral sclerosis (ALS) and potential interventions would be facilitated if motor axon degeneration could be more readily visualized. Here we demonstrate that stimulated Raman scattering (SRS) microscopy could be used to sensitively monitor peripheral nerve degeneration in ALS mouse models and ALS autopsy materials. Three-dimensional imaging of pre-symptomatic SOD1 mouse models and data processing by a correlation-based algorithm revealed that significant degeneration of peripheral nerves could be detected coincidentally with the earliest detectable signs of muscle denervation and preceded physiologically measurable motor function decline...
October 31, 2016: Nature Communications
https://www.readbyqxmd.com/read/27790792/als-associated-endoplasmic-reticulum-proteins-in-denervated-skeletal-muscle-implications-for-motor-neuron-disease-pathology
#11
C M Jesse, E Bushuven, P Tripathi, A Chandrasekar, C M Simon, C Drepper, A Yamoah, A Dreser, I Katona, S Johann, C Beyer, S Wagner, M Grond, S Nikolin, J Anink, D Troost, M Sendtner, A Goswami, J Weis
Alpha-motoneurons and muscle fibers are structurally and functionally interdependent. Both cell types particularly rely on endoplasmic reticulum (ER/SR) functions. Mutations of the ER proteins VAPB, SigR1 and HSP27 lead to hereditary motor neuron diseases (MNDs). Here, we determined the expression profile and localization of these ER proteins/chaperons by immunohistochemistry and immunoblotting in biopsy and autopsy muscle tissue of patients with amyotrophic lateral sclerosis (ALS) and other neurogenic muscular atrophies (NMAs) and compared these patterns to mouse models of neurogenic muscular atrophy...
October 28, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27767211/foxo-dependent-atrogenes-vary-among-catabolic-conditions-and-play-a-key-role-in-muscle-atrophy-induced-by-hindlimb-suspension
#12
Lorenza Brocca, Luana Toniolo, Carlo Reggiani, Roberto Bottinelli, Marco Sandri, Maria Antonietta Pellegrino
Muscle atrophy is a complex process that is in common with many different catabolic diseases including disuse/inactivity and ageing. The signalling pathways that control the atrophy program in the different disuse/inactivity conditions have not yet been completely dissected. It has been recently reported that inhibition of FoxO only partially spared muscle mass after denervation. The purposes of this study were: (i) to determine the involvement of FoxOs in hindlimb suspension disuse model, (ii) to define whether the molecular events of protein breakdown are shared among different unloaded muscles and finally (iii) to compare the data obtained in this model with another model of inactivity such as denervation...
October 21, 2016: Journal of Physiology
https://www.readbyqxmd.com/read/27759889/sarcopenia-age-atrophy-and-myopathy-mitochondrial-oxidative-enzyme-activities
#13
Alan Pestronk, Richard Keeling, Rati Choksi
OBJECTIVE: We studied mitochondrial impairment as a factor in the pathologic equivalent of sarcopenia, muscle fiber atrophy associated with increased age. METHODS: Mitochondrial oxidative enzyme activities and coenzyme Q10 levels were measured in frozen human proximal limb muscles with combined age and atrophy, age alone, atrophy alone, denervation, immune myopathies, and mitochondrial disorders with ophthalmoplegia. RESULTS: Sarcopenia (age and atrophy) had reduced mean activities of mitochondrial Complexes I, II, and II+III, with severe reduction of Complex I activity in 54% of patients...
October 19, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27759887/optimizing-muscle-selection-for-electromyography-in-amyotrophic-lateral-sclerosis
#14
Suma Babu, Erik P Pioro, Jianbo Li, Yuebing Li
INTRODUCTION: We compared the yield of limb and thoracic paraspinal muscles for revealing lower motor neuron (LMN) dysfunction on electromyography (EMG) in amyotrophic lateral sclerosis (ALS). METHODS: A retrospective review of 354 patients with clinically definite or probable ALS was performed. Seventeen limb muscles and thoracic paraspinal muscles were evaluated for the presence of both active and chronic denervation. RESULTS: Distal limb muscles showed the highest electrodiagnostic sensitivities of LMN dysfunction in ALS regardless of onset region and diagnostic certainty at the time of diagnosis...
October 19, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27742296/respiratory-profile-in-patients-after-liver-transplantation
#15
M M C da Silva, R A Corcha, A M Oliveira da Silva, F J B Z Franco, J R de Souza Almeida, I F S F Boin
BACKGROUND: Patients in the immediate post-operative liver transplantation (LxT) period can develop respiratory and functional complications. In the postoperative months, these functions tend to improve. Nevertheless, there are few studies that evaluate precisely and specifically respiratory function in post-LxT long-term after surgery. The objective of the study was to describe the respiratory profile of patients 1 to 6 months after LxT, accompanied by LxT outpatients. METHODS: We included patients between 25 and 60 years old...
September 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27737470/outcomes-of-muscle-reinnervation-with-direct-nerve-implantation-into-the-native-motor-zone-of-the-target-muscle
#16
Stanislaw Sobotka, Jingming Chen, Themba Nyirenda, Liancai Mu
Background Our recent work has demonstrated that the native motor zone (NMZ) within a given skeletal muscle is the best site for muscle reinnervation. This study was designed to explore the outcomes of direct nerve implantation (DNI) into the NMZ of denervated sternomastoid (SM) muscle in a rat model. Methods The right SM muscle was experimentally denervated by transecting its innervating nerve. The proximal stump of the severed SM nerve was immediately implanted into a small muscle slit made in the NMZ of the muscle where denervated motor endplates were concentrated...
October 13, 2016: Journal of Reconstructive Microsurgery
https://www.readbyqxmd.com/read/27725217/neural-network-remodeling-underlying-motor-map-reorganization-induced-by-rehabilitative-training-after-ischemic-stroke
#17
Naohiko Okabe, Takashi Shiromoto, Naoyuki Himi, Feng Lu, Emi Maruyama-Nakamura, Kazuhiko Narita, Nobuhisa Iwachidou, Yoshiki Yagita, Osamu Miyamoto
Motor map reorganization is believed to be one mechanism underlying rehabilitation-induced functional recovery. Although the ipsilesional secondary motor area has been known to reorganize motor maps and contribute to rehabilitation-induced functional recovery, it is unknown how the secondary motor area is reorganized by rehabilitative training. In the present study, using skilled forelimb reaching tasks, we investigated neural network remodeling in the rat rostral forelimb area (RFA) of the secondary motor area during 4weeks of rehabilitative training...
December 17, 2016: Neuroscience
https://www.readbyqxmd.com/read/27717377/immune-response-in-peripheral-axons-delays-disease-progression-in-sod1-g93a-mice
#18
Giovanni Nardo, Maria Chiara Trolese, Giuseppe de Vito, Roberta Cecchi, Nilo Riva, Giorgia Dina, Paul R Heath, Angelo Quattrini, Pamela J Shaw, Vincenzo Piazza, Caterina Bendotti
BACKGROUND: Increasing evidence suggests that the immune system has a beneficial role in the progression of amyotrophic lateral sclerosis (ALS) although the mechanism remains unclear. Recently, we demonstrated that motor neurons (MNs) of C57SOD1(G93A) mice with slow disease progression activate molecules classically involved in the cross-talk with the immune system. This happens a lot less in 129SvSOD1(G93A) mice which, while expressing the same amount of transgene, had faster disease progression and earlier axonal damage...
October 7, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27716425/flank-pseudohernia-following-posterior-rib-fracture-a-case-report
#19
Adam M Butensky, Leah P Gruss, Zachary L Gleit
BACKGROUND: A pseudohernia is an abdominal wall bulge that may be mistaken for a hernia but that lacks the disruption of the abdominal wall that characterizes a hernia. Thus, the natural history and treatment of this condition differ from those of a hernia. This is the first report of a pseudohernia due to cough-associated rib fracture. CASE PRESENTATION: A case of pseudohernia due to fractures of the 10(th) and 11(th) ribs in a 68-year-old white woman is presented...
October 1, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27687289/progression-of-motor-neuron-disease-is-accelerated-and-the-ability-to-recover-is-compromised-with-advanced-age-in-rnls8-mice
#20
Krista J Spiller, Clark R Restrepo, Tahiyana Khan, Anna M Stieber, Linda K Kwong, John Q Trojanowski, Virginia M-Y Lee
In order to treat progressive paralysis in ALS patients, it is critical to develop a mouse that closely models human ALS in both pathology and also in the timing of these events. We have recently generated new TDP-43 bigenic mice (called rNLS8) with doxycycline (Dox)-suppressible expression of human TDP-43 (hTDP-43) harboring a defective nuclear localization signal (hTDP-43∆NLS) under the control of the NEFH promoter. Our previous studies characterized the pathology and disease course in young rNLS8 mice following induction of neuronal hTDP-43ΔNLS...
September 29, 2016: Acta Neuropathologica Communications
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