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https://www.readbyqxmd.com/read/28550268/improving-reproducibility-of-phenotypic-assessments-in-the-dyw-mouse-model-of-laminin-%C3%AE-2-related-congenital-muscular-dystrophy
#1
Raffaella Willmann, Heather Gordish-Dressman, Sarina Meinen, Markus A Rüegg, Qing Yu, Kanneboyina Nagaraju, Ayar Kumar, Mahasweta Girgenrath, Caroline B M Coffey, Vivian Cruz, Pam M Van Ry, Laurent Bogdanik, Cathleen Lutz, Anne Rutkowski, Dean J Burkin
Laminin-α2 related Congenital Muscular Dystrophy (LAMA2-CMD) is a progressive muscle disease caused by partial or complete deficiency of laminin-211, a skeletal muscle extracellular matrix protein. In the last decade, basic science research has queried underlying disease mechanisms in existing LAMA2-CMD murine models and identified possible clinical targets and pharmacological interventions. Experimental rigor in preclinical studies is critical to efficiently and accurately quantify both negative and positive results, degree of efficiency of potential therapeutics and determine whether to move a compound forward for additional preclinical testing...
May 24, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28550176/mechanical-behavior-and-matrisome-gene-expression-in-aneurysm-prone-thoracic-aorta-of-newborn-lysyl-oxidase-knockout-mice
#2
Marius Catalin Staiculescu, Jungsil Kim, Robert P Mecham, Jessica Wagenseil
Mutations in lysyl oxidase (LOX) are associated with thoracic aortic aneurysm and dissection (TAAD). Mice that do not express Lox (Lox(-/-)) die soon after birth and have 60% and 40% reductions in elastin- and collagen-specific crosslinks, respectively. LOX inactivation could also change the expression of secreted factors, structural matrix, and matrix-associated proteins that constitute the aortic matrisome. We hypothesize that the absence of Lox will change the mechanical behavior of the aortic wall due to reduced elastin and collagen crosslinking and alter the expression levels of matrisome and smooth muscle cell (SMC) genes in a vascular location-specific manner...
May 26, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28550175/chronic-hypoxia-attenuates-the-vasodilator-efficacy-of-protein-kinase-g-in-fetal-and-adult-ovine-cerebral-arteries
#3
Richard B Thorpe, Margaret C Hubbell, Jinjutha Silpanisong, James M Williams, William J Pearce
Long-term hypoxia (LTH) attenuates nitric oxide (NO) induced vasorelaxation in ovine middle cerebral arteries. Because cGMP-dependent protein kinase (PKG) is an important mediator of NO signaling in vascular smooth muscle, we tested the hypothesis that LTH diminishes the ability of PKG to interact with target proteins and cause vasorelaxation. Prominent among proteins that regulate vascular tone is the large conductance calcium-sensitive potassium (BK) channel, which is a substrate for PKG and is responsive to phosphorylation on multiple serine/threonine residues...
May 26, 2017: American Journal of Physiology. Heart and Circulatory Physiology
https://www.readbyqxmd.com/read/28549989/overweight-in-elderly-people-induces-impaired-autophagy-in-skeletal-muscle
#4
Yaiza Potes, Beatriz de Luxán-Delgado, Susana Rodriguez-González, Marcela Rodrigues Moreira Guimarães, Juan J Solano, María Fernández-Fernández, Manuel Bermúdez, Jose A Boga, Ignacio Vega-Naredo, Ana Coto-Montes
Sarcopenia is the gradual loss of skeletal muscle mass, strength and quality associated with aging. Changes in body composition, especially in skeletal muscle and fat mass are crucial steps in the development of chronic diseases. We studied the effect of overweight on skeletal muscle tissue in elderly people without reaching obesity to prevent this extreme situation. Overweight induces a progressive protein breakdown reflected as a progressive withdrawal of anabolism against the promoted catabolic state leading to muscle wasting...
May 23, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28549591/single-allele-lmbrd1-knockout-results-in-cardiac-hypertrophy
#5
Linda Tzu-Ling Tseng, Chieh-Liang Lin, Kuei-Hsiang Pan, Kai-Yuan Tzen, Ming-Jai Su, Chia-Ti Tsai, Yi-Han Li, Pai-Chi Li, Fu-Tien Chiang, Shin C Chang, Ming-Fu Chang
BACKGROUND/PURPOSE: LMBD1 protein, a type IV-B plasma membrane protein possessing nine putative trans-membrane domains, was previously demonstrated at cellular level to play a critical part in the signaling cascade of insulin receptor through its involvement in regulating clathrin-mediated endocytosis. However, at physiological level, the significance of LMBD1 protein in cardiac development remains unclear. METHODS: To understand the role of Lmbrd1 gene involved in the cardiac function, heterozygous knockout mice were used as an animal model system...
May 23, 2017: Journal of the Formosan Medical Association, Taiwan Yi Zhi
https://www.readbyqxmd.com/read/28549558/the-kv1-3-channel-blocker-vm24-enhances-muscle-glucose-transporter-4-mobilization-but-does-not-reduce-body-weight-gain-in-diet-induced-obese-male-rats
#6
Lorraine Jaimes-Hoy, Georgina B Gurrola, Miguel Cisneros, Patricia Joseph-Bravo, Lourival D Possani, Jean-Louis Charli
AIMS: Voltage-gated potassium channels 1.3 (Kv1.3) can be targeted to reduce diet-induced obesity and insulin resistance in mice. Since species-specific differences in Kv1.3 expression and pharmacology have been observed, we tested the effect of Vm24, a high-affinity specific blocker of Kv1.3 channels from Vaejovis mexicanus smithi, on body weight (BW), glucose tolerance and insulin resistance in diet-induced obese rats. MATERIALS AND METHODS: Young adult male Wistar rats were switched to a high-fat/high-fructose (HFF) diet...
May 23, 2017: Life Sciences
https://www.readbyqxmd.com/read/28549557/aberrant-distribution-of-the-trochlear-nerve-a-cadaveric-study-supported-by-immunohistochemistry
#7
Kojiro Takezawa, Grant Townsend, Jim Manavis, Mounir Ghabriel
The trochlear nerve is generally considered to be a purely motor nerve supplying one extraocular muscle, the superior oblique. In the current study, 28 orbits were dissected and in one orbit (3.6%), the trochlear nerve divided into two main branches. The medial branch followed the classical course, entered the superior oblique muscle and was presumed to be motor in function. However, before entering the muscle, it partially fused with the frontal nerve, and gave a bundle of nerve fibres to the frontal nerve...
May 23, 2017: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
https://www.readbyqxmd.com/read/28549449/fatal-familial-insomnia-with-abnormal-signals-on-routine-mri-a-case-report-and-literature-review
#8
Tingting Lu, Yuhang Pan, Lisheng Peng, Feng Qin, Xiaobo Sun, Zhengqi Lu, Wei Qiu
BACKGROUND: Fatal familial insomnia (FFI) is a rare autosomal dominant disease caused by the PRNP D178N/129 M mutation. Routine brain CT and MRI usually reveal non-specific features. We report a patient with FFI presenting with diffuse abnormal signals on MRI, later confirmed as combined with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). CASE PRESENTATION: The patient was a 58-year-old female, whose main clinical manifestations were insomnia, movement disorders, autonomic hyperactivity and mental deterioration...
May 26, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28549098/high-protein-foods-and-physical-activity-protect-against-age-related-muscle-loss-and-functional-decline
#9
M Loring Bradlee, Jabed Mustafa, Martha R Singer, Lynn L Moore
Background: Some clinical trials suggest that protein supplementation enhances the effects of resistance exercise on skeletal muscle mass (SMM); fewer studies examine the effects of diets rich in protein-source foods on SMM and functional status among community-dwelling adults. Methods: Data from the Framingham Offspring study including diet (three-day records, exams 3 and 5), physical activity (exams 2 and 4), percent SMM (%SMM) (exams 6 and 7), and functional performance (exams 5 through 8) were used to evaluate independent and combined effects of physical activity and high-protein foods on adjusted mean %SMM (using analysis of covariance) and risk of functional decline (using Cox proportional hazard's models)...
May 25, 2017: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/28548960/severe-hypertriglyceridemia-due-to-two-novel-loss-of-function-lipoprotein-lipase-gene-mutations-c310r-e396v-in-a-chinese-family-associated-with-recurrent-acute-pancreatitis
#10
Yu Lun, Xiaofang Sun, Ping Wang, Jingwei Chi, Xu Hou, Yangang Wang
Lipoprotein lipase (LPL) is widely expressed in skeletal muscles, cardiac muscles as well as adipose tissue and involved in the catabolism of triglyceride. Herein we have systematically characterized two novel loss-of-function mutations in LPL from a Chinese family in which afflicted members were manifested by severe hypertriglyceridemia and recurrent pancreatitis. DNA sequencing revealed that the proband was a heterozygote carrying a novel c.T928C (p.C310R) mutation in exon 6 of the LPL gene. Another member of the family was detected to be a compound heterozygote who along with the c...
May 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/28548864/oral-administration-of-a-medium-containing-both-d-aspartate-producing-live-bacteria-and-d-aspartate-reduces-rectal-temperature-in-chickens
#11
P H Do, P V Tran, M A Bahry, H Yang, G Han, A Tsuchiya, Y Asami, M Furuse, V S Chowdhury
1. The aim of this study was to investigate the effects on the rectal temperature of young chickens of the oral administration of a medium that contained both live bacteria that produce D-aspartate (D-Asp) and D-Asp. 2. In Experiment 1, chickens were subjected to chronic oral administration of either the medium (containing live bacteria and 2.46 μmol D-Asp) or water from 7 to 14 d of age. Plasma-free amino acids as well as mitochondrial biogenic gene expression in the breast muscle were analysed. In Experiment 2, 7-d-old chickens were subjected to acute oral administration of the above medium or of an equimolar amount of D-Asp to examine their effect on changes in rectal temperature...
May 26, 2017: British Poultry Science
https://www.readbyqxmd.com/read/28548451/molecular-and-functional-characterization-of-the-american-cockroach-periplaneta-americana-rab5-the-first-exopterygotan-low-molecular-weight-ovarian-gtpase-during-oogenesis
#12
Mohamed Elmogy, Amr A Mohamed, Muhammad Tufail, Tomohide Uno, Makio Takeda
The small Rab GTPases are key regulators of membrane vesicle trafficking. Ovaries of Periplaneta americana (Linnaeus) (Blattodea: Blattidae) have small molecular weight GTP/ATP-binding proteins during early and late vitellogenic periods of oogenesis. However, the identification and characterization of the detected proteins have not been yet reported. Herein, we cloned a cDNA encoding Rab5 from the American cockroach, P. americana, ovaries (PamRab5). It comprises 796 bp, encoding a protein of 213 amino acid residues with a predicted molecular weight of 23...
May 26, 2017: Insect Science
https://www.readbyqxmd.com/read/28546635/local-cryotherapy-minimally-impacts-the-metabolome-and-transcriptome-of-human-skeletal-muscle
#13
Dylan C Sarver, Kristoffer B Sugg, Nathaniel P Disser, Elizabeth R Sibilsky Enselman, Tariq M Awan, Christopher L Mendias
Cryotherapy is commonly used in the treatment of skeletal muscle injuries. However, the data to support the use of cryotherapy is inconclusive, and the biochemical etiology of cryotherapy in human skeletal muscle remains largely unknown. We therefore sought to determine how a clinically-relevant dose of cryotherapy would impact the transcriptome and metabolome of skeletal muscle. Eight healthy male subjects (age 24.7 ± 4.5 years, BMI 22.2 ± 1.6) received a 15 minute bout of local cryotherapy, delivered via ice cup massage over the anterolateral thigh...
May 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28546468/influence-of-maximal-fat-oxidation-on-long-term-weight-loss-maintenance-in-humans
#14
Sune Dandanell, Karina Husted, Signe Amdisen, Andreas Vigelsø, Flemming Dela, Steen Larsen, Jorn Wulff Helge
Impaired maximal fat oxidation has been linked to obesity and weight regain after weight loss. The aim was to investigate the relationship between maximal fat oxidation (MFO) and long-term weight loss maintenance. Eighty subjects (mean(SD)) (Age: 36(13) yrs; BMI: 38(1) kg/m(2)) were recruited from a total of 2420 former participants of an 11-12-week lifestyle intervention. Three groups were established based on % weight loss at follow-up (5.3(3.3) yrs): Clinical weight loss maintenance (CWL) >10% weight loss, moderate weight loss (MWL) 1-10%, and weight regain (WR)...
May 25, 2017: Journal of Applied Physiology
https://www.readbyqxmd.com/read/28546457/amino-acid-homeostasis-and-signalling-in-mammalian-cells-and-organisms
#15
REVIEW
Stefan Bröer, Angelika Bröer
Cells have a constant turnover of proteins that recycle most amino acids over time. Net loss is mainly due to amino acid oxidation. Homeostasis is achieved through exchange of essential amino acids with non-essential amino acids and the transfer of amino groups from oxidised amino acids to amino acid biosynthesis. This homeostatic condition is maintained through an active mTORC1 complex. Under amino acid depletion, mTORC1 is inactivated. This increases the breakdown of cellular proteins through autophagy and reduces protein biosynthesis...
May 25, 2017: Biochemical Journal
https://www.readbyqxmd.com/read/28546310/heterozygous-gnal-mice-are-a-novel-animal-model-to-study-dystonia-pathophysiology
#16
Assunta Pelosi, Fabien Menardy, Daniela Popa, Jean-Antoine Girault, Denis Hervé
Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions. Its pathophysiological mechanisms are still poorly understood. Dominant mutations of the GNAL gene are a cause of isolated dystonia (DYT25) in patients. Some mutations result in a complete loss of function of the encoded protein, Gαolf, an adenylyl-cyclase-stimulatory G protein highly enriched in striatal projection neurons, in which it mediates the actions of dopamine and adenosine. We used male and female heterozygous Gnal knockout mice (Gnal+/-) to study how GNAL haplodeficiency is implicated in dystonia...
May 25, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28546288/dynamic-changes-in-the-skeletal-muscle-proteome-during-denervation-induced-atrophy
#17
Franziska Lang, Sriram Aravamudhan, Hendrik Nolte, Clara Tuerk, Soraya Hölper, Stefan Müller, Stefan Günther, Bert Blaauw, Thomas Braun, Marcus Krüger
Loss of neuronal stimulation enhances protein breakdown and reduces protein synthesis, causing rapid muscle mass loss. To elucidate the pathophysiological adaptations that occur in atrophying muscles, we used stable isotope labelling and mass spectrometry to accurately quantify protein expression changes during denervation-induced atrophy after sciatic nerve section in the mouse gastrocnemius muscle (GAST). Additionally, mice were fed a SILAC diet containing (13)C6 lysine for four, seven, or eleven days to calculate relative levels of protein synthesis in denervated and control muscles...
May 25, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28546218/adamts5-deficiency-in-calcified-aortic-valves-is-associated-with-elevated-pro-osteogenic-activity-in-valvular-interstitial-cells
#18
Fei Li, Rui Song, Lihua Ao, T Brett Reece, Joseph C Cleveland, Nianguo Dong, David A Fullerton, Xianzhong Meng
OBJECTIVE: Extracellular matrix proteinases are implicated in the pathogenesis of calcific aortic valve disease. The ADAMTS5 (a disintegrin and metalloproteinase with thrombospondin motifs 5) enzyme is secreted, matrix-associated metalloendopeptidase, capable of degrading extracellular matrix proteins, particularly matrilin 2. We sought to determine the role of the ADAMTS5/matrilin 2 axis in mediating the phenotype transition of valvular interstitial cells (VICs) associated with calcific aortic valve disease...
May 25, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28546027/molecular-characterization-of-two-distinct-smads-gene-and-their-roles-in-the-response-to-bacteria-change-and-wound-healing-from-hyriopsis-cumingii
#19
Baoqing Hu, Peipei Yi, Zhenfang Li, Ming Zhang, Chungen Wen, Shaoqing Jian, Gang Yang
The proteins of Smad family are critical components of the TGF-β superfamily signal pathway. In this paper, we cloned two intracellular mediators of TGF-β signaling, Smad3 and Smad5, from the pearl mussel Hyriopsis cumingii. The full length cDNA of HcSmad3 and HcSmad5 were 2052 bp and 1908 bp and encoded two polypeptides of 418 and 461amino acid residues, respectively. The deduced amino acid of HcSmad3 and HcSmad5 possessed two putative conserved domains, MH1 and MH2, a conserved phosphorylation motif SSXS at the carboxyl-terminal...
May 22, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28545905/resveratrol-alleviates-oxidative-damage-in-enteric-neurons-and-associated-gastrointestinal-dysfunction-caused-by-chemotherapeutic-agent-oxaliplatin
#20
Elizabeth L Donald, Lily Stojanovska, Vasso Apostolopoulos, Kulmira Nurgali
Oxaliplatin is a first-line chemotherapeutic agent used for the treatment of colorectal cancer. Its use is associated with severe gastrointestinal (GI) side-effects, associated with oxidative damage and neurotoxicity to the enteric neurons. Resveratrol is a potent anti-oxidant that has been shown to protect against oxidative damage and neurotoxicity in other neurons and could therefore prevent oxaliplatin-induced damage to enteric neurons. We determined whether co-administration of resveratrol with oxaliplatin alleviates enteric neuron toxicity and GI dysfunction in mice...
May 17, 2017: Maturitas
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