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C1-2 dysfunction

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https://www.readbyqxmd.com/read/29346563/lc3-immunostaining-in-the-inferior-olivary-nuclei-of-cats-with-niemann-pick-disease-type-c1-is-associated-with-patterned-purkinje-cell-loss
#1
Brittney L Gurda, Jessica H Bagel, Samantha J Fisher, Mark L Schultz, Andrew P Lieberman, Peter Hand, Charles H Vite, Gary P Swain
The feline model of Niemann-Pick disease, type C1 (NPC1) recapitulates the clinical, neuropathological, and biochemical abnormalities present in children with NPC1. The hallmarks of disease are the lysosomal storage of unesterified cholesterol and multiple sphingolipids in neurons, and the spatial and temporal distribution of Purkinje cell death. In feline NPC1 brain, microtubule-associated protein 1 light chain 3 (LC3) accumulations, indicating autophagosomes, were found within axons and presynaptic terminals...
January 13, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29303389/a-modification-of-laparoscopic-type-c1-hysterectomy-to-reduce-postoperative-bladder-dysfunction-a-retrospective-study
#2
Wei Jiang, Meirong Liang, Douxing Han, Hui Liu, Ling Li, Meiling Zhong, Lin Luo, Siyuan Zeng
OBJECTIVE: To investigate the feasibility of a modified type C1 hysterectomy, using the deep uterine vein (DUV) and its branches as an anatomical landmark, to reduce postoperative bladder dysfunction in cervical cancer patients. METHODS: One hundred fifty-two stage IA2-IIB cervical cancer patients were enrolled to undergo a laparoscopic hysterectomy. According to the operation methods, 93 or 59 of the patients were assigned to a type C1 hysterectomy (group 1), using the DUV and its branches as an anatomical landmark, and a type C2 hysterectomy (group 2), respectively...
January 5, 2018: Journal of Investigative Surgery: the Official Journal of the Academy of Surgical Research
https://www.readbyqxmd.com/read/29187724/in-depth-insight-into-the-mechanisms-of-cardiac-dysfunction-in-patients-with-childhood-cancer-after-anthracycline-treatment-using-layer-specific-strain-analysis
#3
Kana Yazaki, Ken Takahashi, Sachie Shigemitsu, Mariko Yamada, Takeshi Iso, Maki Kobayashi, Katsumi Akimoto, Hiroyuki Tamaichi, Junya Fujimura, Masahiro Saito, Masaki Nii, Toshiaki Shimizu
BACKGROUND: Anthracycline cardiotoxicity affects clinical outcomes, and its early detection using methods that rely on conventional echocardiography, such as left ventricular ejection fraction (LVEF) is difficult. This study aimed to evaluate the characteristics and the differences in cardiac dysfunction among childhood cancer survivors in 3 age groups using layer-specific strain analysis in a wide age range.Methods and Results:The 56 patients (median age: 15 [range: 6.8-40.2] years) who had been treated with anthracycline for childhood cancer were divided into 3 age groups (C1: 6-12 years, C2: 13-19 years, C3: 20-40 years) after anthracycline treatment, and 72 controls of similar ages were divided into 3 corresponding groups (N1, N2, and N3)...
November 29, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29184713/posterior-atlantoaxial-fusion-using-a-c2-transverse-foramen-penetrating-screw-a-technical-note
#4
Yasuhiro Takeshima, Hideki Shigematsu, Kengo Konishi, Ichiro Nakagawa, Yasushi Motoyama, Hiroyuki Nakase
Background: Although recent development of screw instrumentation techniques for rigid fixation of the atlantoaxial joint has increased surgical options, patients in whom screws of any type cannot be safely placed are sometimes encountered. We present a unique surgical technique for C1-2 transarticular screw placement utilizing a novel trajectory. Methods: A 35-year-old male with a history of Down's syndrome and cognitive dysfunction with hyperkinesis spontaneously developed rapid onset of tetraparesis and gait disturbance...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/29169960/oxytocin-inhibits-the-rat-medullary-dorsal-horn-sp5c-c1-nociceptive-transmission-through-ot-but-not-v1a-receptors
#5
Enrique García-Boll, Guadalupe Martínez-Lorenzana, Miguel Condés-Lara, Abimael González-Hernández
The medullary dorsal horn (MDH or Sp5c/C1 region) plays a key role modulating the nociceptive input arriving from craniofacial structures. Some reports suggest that oxytocin could play a role modulating the nociceptive input at the MDH level, but no study has properly tested this hypothesis. Using an electrophysiological and pharmacological approach, the present study aimed to determine the effect of oxytocin on the nociceptive signaling in the MDH and the receptor involved. In sevoflurane, anesthetized rats, we performed electrophysiological unitary recordings of second order neurons at the MDH region responding to peripheral nociceptive-evoked responses of the first branch (V1; ophthalmic) of the trigeminal nerve...
November 20, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28945060/benefits-of-micronized-purified-flavonoid-fraction-as-adjuvant-therapy-on-the-inflammatory-response-after-sclerotherapy
#6
Vadim Y Bogachev, Boris V Boldin, Victor N Lobanov
BACKGROUND: Sclerotherapy is the treatment of choice for telangiectasias. The aim of this study was to investigate the systemic and local inflammatory response after sclerotherapy in women with chronic venous disease (CVD) and to assess the effects of micronized purified flavonoid fraction (MPFF) in combination with sclerotherapy on markers of inflammation and endothelial dysfunction. METHODS: 60 women with primary CVD CEAP class C1 were randomly assigned sclerotherapy in combination with MPFF treatment (N=30) or sclerotherapy alone (control) group (N=30)...
September 22, 2017: International Angiology: a Journal of the International Union of Angiology
https://www.readbyqxmd.com/read/28870191/effectiveness-of-a-specific-manual-approach-to-the-suboccipital-region-in-patients-with-chronic-mechanical-neck-pain-and-rotation-deficit-in-the-upper-cervical-spine-study-protocol-for-a-randomized-controlled-trial
#7
Vanessa González Rueda, Carlos López de Celis, Martín Eusebio Barra López, Andoni Carrasco Uribarren, Sara Castillo Tomás, Cesar Hidalgo García
BACKGROUND: Mechanical neck pain is a highly prevalent problem in primary healthcare settings. Many of these patients have restricted mobility of the cervical spine. Several manual techniques have been recommended for restoring cervical mobility, but their effectiveness in these patients is unknown. The aim of the present study is to compare the effectiveness of two types of specific techniques of the upper neck region: the pressure maintained suboccipital inhibition technique (PMSIT) and the translatory dorsal glide mobilization (TDGM) C0-C1 technique, as adjuncts to a protocolized physiotherapy treatment of the neck region in subjects with chronic mechanical neck pain and rotation deficit in the upper cervical spine...
September 5, 2017: BMC Musculoskeletal Disorders
https://www.readbyqxmd.com/read/28670631/cardiac-directed-expression-of-adenylyl-cyclase-catalytic-domain-reverses-cardiac-dysfunction-caused-by-sustained-beta-adrenergic-receptor-stimulation
#8
Mei Hua Gao, N Chin Lai, Dimosthenis Giamouridis, Young Chul Kim, Zhen Tan, Tracy Guo, Wolfgang H Dillmann, Jorge Suarez, H Kirk Hammond
OBJECTIVE: To test the hypothesis that cardiac-directed expression of the cytoplasmic domains of adenylyl cyclase-6 (AC6) would have beneficial effects on the heart. BACKGROUND: Eliminating the two transmembrane domains of AC6 yields a protein with an intact catalytic domain that is disengaged from membrane-associated β-adrenergic receptor stimulation, but with enhanced propensity for intracellular interactions. METHODS: We constructed a peptide of the C1 and C2 segments of AC6 (C1C2), expressed C1C2 in an adenovirus vector and generated transgenic lines with cardiac-directed C1C2 expression, which underwent sustained isoproterenol (Iso) infusion...
December 2016: JACC. Basic to Translational Science
https://www.readbyqxmd.com/read/28662932/dynamic-in-vivo-3d-atlantoaxial-spine-kinematics-during-upright-rotation
#9
William Anderst, Bryan Rynearson, Tyler West, William Donaldson, Joon Lee
Diagnosing dysfunctional atlantoaxial motion is challenging given limitations of current diagnostic imaging techniques. Three-dimensional imaging during upright functional motion may be useful in identifying dynamic instability not apparent on static imaging. Abnormal atlantoaxial motion has been linked to numerous pathologies including whiplash, cervicogenic headaches, C2 fractures, and rheumatoid arthritis. However, normal C1/C2 rotational kinematics under dynamic physiologic loading have not been previously reported owing to imaging difficulties...
June 20, 2017: Journal of Biomechanics
https://www.readbyqxmd.com/read/28621447/metformin-prevents-vascular-prostanoid-release-alterations-induced-by-a-high-fat-diet-in-rats
#10
H J Lee, S M Cantú, A S Donoso, M R Choi, H A Peredo, A M Puyó
Perivascular adipose tissue dysfunction induced by high-fat feeding leads to alterations in the modulation of inflammation, contractile activity of the vascular smooth muscle and endothelial function, all risk factors in the development of hypertension. Metformin, an activator of AMP-activated protein kinase (AMPK), is currently the first-line drug treatment for type 2 diabetes (T2DM) and metabolic syndrome. Besides its glucose-lowering effect, there is an interest in actions of this drug with potential relevance in cardiovascular diseases...
June 2017: Autonomic & Autacoid Pharmacology
https://www.readbyqxmd.com/read/28415010/structure-and-biological-properties-of-mixed-ligand-cu-ii-schiff-base-complexes-as-potential-anticancer-agents
#11
Yi Gou, Jinlong Li, Boyi Fan, Bohui Xu, Min Zhou, Feng Yang
We synthesized two mixed-ligand Cu(II) complexes containing different aroylhydrazone ligands and a pyridine co-ligand, namely, [Cu(L1)(Py)] (C1) and [Cu(L2)(Py)(Br)] (C2) (L1 = (E)-2-hydroxy-N'-((2-hydroxynaphthalen-1-yl)methylene)benzohydrazide, Py = pyridine, L2 = (E)-2-hydroxy-N'-(phenyl(pyridin-2-yl)methylene)benzohydrazide), and assessed their chemical and biological properties to understand their marked activity. C2 showed better anticancer activity than C1 in various human cancer cell lines, including the cisplatin-resistant lung cancer cell line A549cisR...
July 7, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28328347/prevention-of-hereditary-angioedema-attacks-with-a-subcutaneous-c1-inhibitor
#12
RANDOMIZED CONTROLLED TRIAL
Hilary Longhurst, Marco Cicardi, Timothy Craig, Konrad Bork, Clive Grattan, James Baker, Huamin H Li, Avner Reshef, James Bonner, Jonathan A Bernstein, John Anderson, William R Lumry, Henriette Farkas, Constance H Katelaris, Gordon L Sussman, Joshua Jacobs, Marc Riedl, Michael E Manning, Jacques Hebert, Paul K Keith, Shmuel Kivity, Sergio Neri, Donald S Levy, Maria L Baeza, Robert Nathan, Lawrence B Schwartz, Teresa Caballero, William Yang, Ioana Crisan, María D Hernandez, Iftikhar Hussain, Michael Tarzi, Bruce Ritchie, Pavlina Králíčková, Mar Guilarte, Syed M Rehman, Aleena Banerji, Richard G Gower, Debra Bensen-Kennedy, Jonathan Edelman, Henrike Feuersenger, John-Philip Lawo, Thomas Machnig, Dipti Pawaskar, Ingo Pragst, Bruce L Zuraw
BACKGROUND: Hereditary angioedema is a disabling, potentially fatal condition caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein. In a phase 2 trial, the use of CSL830, a nanofiltered C1 inhibitor preparation that is suitable for subcutaneous injection, resulted in functional levels of C1 inhibitor activity that would be expected to provide effective prophylaxis of attacks. METHODS: We conducted an international, prospective, multicenter, randomized, double-blind, placebo-controlled, dose-ranging, phase 3 trial to evaluate the efficacy and safety of self-administered subcutaneous CSL830 in patients with type I or type II hereditary angioedema who had had four or more attacks in a consecutive 2-month period within 3 months before screening...
March 23, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28260735/mechanisms-of-left-ventricular-dysfunction-assessed-by-layer-specific-strain-analysis-in-patients-with-repaired-tetralogy-of-fallot
#13
Mariko Yamada, Ken Takahashi, Maki Kobayashi, Kana Yazaki, Hirobumi Takayasu, Katsumi Akimoto, Masahiko Kishiro, Akio Inage, Tadahiro Yoshikawa, In-Sam Park, Keisuke Nakanishi, Shiori Kawasaki, Toshiaki Shimizu
BACKGROUND: Left ventricular (LV) dysfunction in patients with repaired tetralogy of Fallot (rTOF) is an important risk factor for adverse outcomes. The aim of this study was to assess the details and time course of such LV dysfunction using layer-specific strain analysis by echocardiography.Methods and Results:The 66 patients with rTOF (mean age, 16.3±9.3 years) were divided into 3 groups (T1: 4-10 years, T2: 11-20 years, T3: 21-43 years), and 113 controls of similar age (mean age, 17...
March 3, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/27923782/comparing-arterial-function-parameters-for-the-prediction-of-coronary-heart-disease-events-the-multi-ethnic-study-of-atherosclerosis-mesa
#14
Elizabeth K Hom, Daniel A Duprez, David R Jacobs, David A Bluemke, Lyndia C Brumback, Joseph F Polak, Carmen A Peralta, Philip Greenland, Sheryl L Magzamen, João A C Lima, Alban Redheuil, David M Herrington, James H Stein, Dhananjay Vaidya, Pamela Ouyang, Joel D Kaufman
Arterial dysfunction has been linked to decline in cardiac function and increased risk of cardiovascular disease events. We calculated the value of arterial function, measured at baseline (2000-2002), in predicting time to first coronary heart disease (CHD) event (median follow-up, 10.2 years) among participants in the Multi-Ethnic Study of Atherosclerosis (MESA). Measures included the following: C1 and C2, derived from diastolic pulse contour analysis from the radial artery blood pressure waveform obtained by tonometry (n = 6,336); carotid distensibility and Young's elastic modulus at the carotid artery, derived from carotid artery ultrasonography (n = 6,531 and 6,528); and aortic distensibility, measured using cardiac magnetic resonance imaging (n = 3,677)...
December 15, 2016: American Journal of Epidemiology
https://www.readbyqxmd.com/read/27769893/phenylethanolamine-n-methyltransferase-gene-expression-in-adrenergic-neurons-of-spontaneously-hypertensive-rats
#15
Julie Grandbois, Sandhya Khurana, Kelly Graff, Phong Nguyen, Leah Meltz, T C Tai
Epinephrine is synthesised by the catecholamine biosynthetic enzyme, phenylethanolamine N-methyltransferase (PNMT), primarily in chromaffin cells of the adrenal medulla and secondarily in brainstem adrenergic neurons of the medulla oblongata. Epinephrine is an important neurotransmitter/neurohormone involved in cardiovascular regulation; however, overproduction is detrimental with negative outcomes such as cellular damage, cardiovascular dysfunction, and hypertension. Genetic mapping studies have linked elevated expression of PNMT to hypertension...
December 2, 2016: Neuroscience Letters
https://www.readbyqxmd.com/read/27586038/developmental-delay-in-motor-skill-acquisition-in-niemann-pick-c1-mice-reveals-abnormal-cerebellar-morphogenesis
#16
Paola Caporali, Francesco Bruno, Giampiero Palladino, Jessica Dragotto, Laura Petrosini, Franco Mangia, Robert P Erickson, Sonia Canterini, Maria Teresa Fiorenza
Niemann-Pick type C1 (NPC1) disease is a lysosomal storage disorder caused by defective intracellular trafficking of exogenous cholesterol. Purkinje cell (PC) degeneration is the main sign of cerebellar dysfunction in both NPC1 patients and animal models. It has been recently shown that a significant decrease in Sonic hedgehog (Shh) expression reduces the proliferative potential of granule neuron precursors in the developing cerebellum of Npc1 (-/-) mice. Pursuing the hypothesis that this developmental defect translates into functional impairments, we have assayed Npc1-deficient pups belonging to the milder mutant mouse strain Npc1 (nmf164) for sensorimotor development from postnatal day (PN) 3 to PN21...
September 1, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27559458/clinical-presentation-of-cervical-myelopathy-at-c1-2-level
#17
Yasutaka Murahashi, Tsuneo Takebayashi, Yoshinori Terashima, Hajime Tsuda, Mitsunori Yoshimoto, Toshihiko Yamashita
STUDY DESIGN: Single-center retrospective study. PURPOSE: To clarify the clinical features of cervical myelopathy at the C1-2 level. OVERVIEW OF LITERATURE: Methods for distinguishing the affected level based on myelomere symptoms or dysfunction of the conducting pathway were established. However, no symptoms have been identified as being specific to the C1-2 level segment. METHODS: We evaluated 24 patients with cervical myelopathy due to spinal cord compression at the C1-2 level...
August 2016: Asian Spine Journal
https://www.readbyqxmd.com/read/27554845/a-novel-role-for-small-molecule-glycomimetics-in-the-protection-against-lipid-induced-endothelial-dysfunction-involvement-of-akt-enos-and-nrf2-are-signaling
#18
Ayman M Mahmoud, Fiona L Wilkinson, Alan M Jones, James A Wilkinson, Miguel Romero, Juan Duarte, M Yvonne Alexander
BACKGROUND: Glycomimetics are a diverse array of saccharide-inspired compounds, designed to mimic the bioactive functions of glycosaminoglycans. Therefore, glycomimetics represent a unique source of novel therapies to target aberrant signaling and protein interactions in a wide range of diseases. We investigated the protective effects of four newly synthesized small molecule glycomimetics against lipid-induced endothelial dysfunction, with an emphasis on nitric oxide (NO) and oxidative stress...
January 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27499185/serological-evidence-of-infection-with-rodent-borne-hepatitis-e-virus-hev-c1-or-antigenically-related-virus-in-humans
#19
Kenta Shimizu, Sugihiro Hamaguchi, Cuong Chi Ngo, Tian-Cheng Li, Shuji Ando, Kumiko Yoshimatsu, Shumpei P Yasuda, Takaaki Koma, Rie Isozumi, Yoshimi Tsuda, Hiromi Fujita, Thuy Thanh Pham, Mai Quynh LE, Anh Duc Dang, Tuan Quang Nguyen, Lay-Myint Yoshida, Koya Ariyoshi, Jiro Arikawa
Zoonotic potential of a rat-derived hepatitis E virus (HEV), designated as HEV-C1, remains unknown. To evaluate the risk for HEV-C1 infection in humans, paired sera of 208 hospitalized febrile patients collected from 2001 to 2003 in Hanoi, Vietnam, were examined for IgG antibodies to HEV-C1 and genotype 1 HEV (HEV-1), which is common in humans. IgG antibodies to virus-like particles (VLPs) of HEV-C1 and/or HEV-1 were detected from 99 of the 208 convalescent sera in enzyme-linked immunosorbent assay (ELISA)...
December 1, 2016: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/27307499/cerebrospinal-fluid-calbindin-d-concentration-as-a-biomarker-of-cerebellar-disease-progression-in-niemann-pick-type-c1-disease
#20
Allison Bradbury, Jessica Bagel, Maureen Sampson, Nicole Farhat, Wenge Ding, Gary Swain, Maria Prociuk, Patricia O'Donnell, Kenneth Drobatz, Brittney Gurda, Christopher Wassif, Alan Remaley, Forbes Porter, Charles Vite
Niemann-Pick type C (NPC) 1 disease is a rare, inherited, neurodegenerative disease. Clear evidence of the therapeutic efficacy of 2-hydroxypropyl-β-cyclodextrin (HPβCD) in animal models resulted in the initiation of a phase I/IIa clinical trial in 2013 and a phase IIb/III trial in 2015. With clinical trials ongoing, validation of a biomarker to track disease progression and serve as a supporting outcome measure of therapeutic efficacy has become compulsory. In this study, we evaluated calcium-binding protein calbindin D-28K (calbindin) concentrations in the cerebrospinal fluid (CSF) as a biomarker of NPC1 disease...
August 2016: Journal of Pharmacology and Experimental Therapeutics
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