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Inclusion body disease

Alessandro Mantovani, Lucia Mingolla, Riccardo Rigolon, Isabella Pichiri, Valentina Cavalieri, Giacomo Zoppini, Giuseppe Lippi, Enzo Bonora, Giovanni Targher
BACKGROUND: Recent studies suggested that nonalcoholic fatty liver disease (NAFLD) is associated with an increased prevalence of cardiovascular disease (CVD) in type 1 diabetes. We assessed whether NAFLD also predicts the risk of incident CVD events in type 1 diabetic adults. METHODS: We studied a retrospective cohort of 286 type 1 diabetic outpatients (mean age 43±14years; median duration of diabetes 17 [10-30] years) without secondary causes of chronic liver diseases, who were followed for a mean period of 5...
October 17, 2016: International Journal of Cardiology
Oznur Bayraktar, Ozlem Oral, Nur Mehpare Kocaturk, Yunus Akkoc, Karin Eberhart, Ali Kosar, Devrim Gozuacik
The ubiquitin-proteasome system (UPS) degrades soluble proteins and small aggregates, whereas macroautophagy (autophagy herein) eliminates larger protein aggregates, tangles and even whole organelles in a lysosome-dependent manner. VCP/p97 was implicated in both pathways. VCP/p97 mutations cause a rare multisystem disease called IBMPFD (Inclusion Body Myopathy with Paget's Disease and Frontotemporal Dementia). Here, we studied the role IBMPFD-related mutants of VCP/p97 in autophagy. In contrast with the wild-type VCP/p97 protein or R155C or R191Q mutants, the P137L mutant was aggregate-prone...
2016: PloS One
James C Campbell, Alexander Tischer, Venkata Machha, Laurie Moon-Tasson, Banumathi Sankaran, Choel Kim, Matthew Auton
von Willebrand factor׳s (VWF) primary hemostatic responsibility is to deposit platelets at sites of vascular injury to prevent bleeding. This function is mediated by the interaction between the VWF A1 domain and the constitutively active platelet receptor, GPIbα. The crystal structure of the A1 domain harboring the von Willebrand disease (vWD) type 2M mutation p.Gly1324Ser has been recently published in the Journal of Biological Chemistry describing its effect on the function and structural stability of the A1 domain of VWF, "Mutational constraints on local unfolding inhibit the rheological adaptation of von Willebrand factor" [1]...
June 2016: Data in Brief
Massimiliano Ruscica, Chiara Pavanello, Sara Gandini, Monica Gomaraschi, Cecilia Vitali, Chiara Macchi, Beatrice Morlotti, Gilda Aiello, Raffaella Bosisio, Laura Calabresi, Anna Arnoldi, Cesare R Sirtori, Paolo Magni
BACKGROUND: Cardiovascular diseases are currently the commonest cause of death worldwide. Different strategies for their primary prevention have been planned, taking into account the main known risk factors, which include an atherogenic lipid profile and visceral fat excess. METHODS: The study was designed as a randomized, parallel, single-center study with a nutritional intervention duration of 12 weeks. Whole soy foods corresponding to 30 g/day soy protein were given in substitution of animal foods containing the same protein amount...
October 18, 2016: European Journal of Nutrition
Fan Meng, Hao Yang, Colin Jack, Huaqun Zhang, Abraham Moller, Devin Spivey, Richard C Page, David L Tierney, Michael W Crowder
Matrix metalloproteinase 7 (MMP7/matrilysin-1) has been implicated in many pathological conditions, such as in cancer and inflammatory diseases; therefore, MMP7 has been targeted for drugs. Success in developing a clinical inhibitor, which exhibits suitable specificity and selectivity, will likely require structural and/or kinetic evaluation of enzyme/inhibitor interactions. To enable these future studies we herein describe the over-expression, purification, and characterization of the catalytic domain of MMP7 (cdMMP7)...
October 14, 2016: Journal of Inorganic Biochemistry
Linlu Zhao, Darren M Roffey, Suzan Chen
STUDY DESIGN: Systematic review and meta-analysis. OBJECTIVE: To assess and synthesize the current evidence on the association between the rs1256120 single nucleotide polymorphism (SNP) of the estrogen receptor beta gene (ESR2) and adolescent idiopathic scoliosis (AIS). SUMMARY OF BACKGROUND DATA: Hormonal disturbance has been postulated as a potential etiological factor in the development of AIS. As estrogen receptors are important mediators of estrogen response, mutations in these genes, including rs1256120 of ESR2, have been chosen as susceptibility candidates for AIS predisposition...
October 17, 2016: Spine
Chrisovalantis Papadopoulos, Philipp Kirchner, Monika Bug, Daniel Grum, Lisa Koerver, Nina Schulze, Robert Poehler, Alina Dressler, Sven Fengler, Khalid Arhzaouy, Vanda Lux, Michael Ehrmann, Conrad C Weihl, Hemmo Meyer
Rupture of endosomes and lysosomes is a major cellular stress condition leading to cell death and degeneration. Here, we identified an essential role for the ubiquitin-directed AAA-ATPase, p97, in the clearance of damaged lysosomes by autophagy. Upon damage, p97 translocates to lysosomes and there cooperates with a distinct set of cofactors including UBXD1, PLAA, and the deubiquitinating enzyme YOD1, which we term ELDR components for Endo-Lysosomal Damage Response. Together, they act downstream of K63-linked ubiquitination and p62 recruitment, and selectively remove K48-linked ubiquitin conjugates from a subpopulation of damaged lysosomes to promote autophagosome formation...
October 17, 2016: EMBO Journal
Ramon Vilallonga, José Manuel Moreno Villares, Diego Yeste Fernández, Raquel Sánchez Santos, Felipe Casanueva Freijo, Francisco Santolaya Ochando, Nuria Leal Hernando, Albert Lecube Torelló, Luis Antonio Castaño González, Albert Feliu, Gontrand Lopez-Nava, Dolores Frutos, Antonio J Torres Garcia, Juan Carlos Ruiz de Adana
PURPOSE: In recent years, the incidence of childhood obesity in Europe, and Spain in particular, has increased dramatically. Bariatric surgery could play a major role in treating of adolescents with severe obesity. However, no specific guidelines for bariatric surgery currently exist in Spain. METHODS: The Board of the Spanish Society for Obesity Surgery and Metabolic Diseases (SECO) proposed a study of childhood obesity by using the Delphi method. This prospective study involved 60 experts from nine national societies...
October 17, 2016: Obesity Surgery
Thomas Schwartz, Louise Pyndt Diederichsen, Ingrid E Lundberg, Ivar Sjaastad, Helga Sanner
Idiopathic inflammatory myopathies (IIM) include the main subgroups polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM) and juvenile DM (JDM). The mentioned subgroups are characterised by inflammation of skeletal muscles leading to muscle weakness and other organs can also be affected as well. Even though clinically significant heart involvement is uncommon, heart disease is one of the major causes of death in IIM. Recent studies show an increased prevalence of traditional cardiovascular risk factors in JDM and DM/PM, which need attention...
2016: RMD Open
Mansi R Khanna, Jane Kovalevich, Virginia M-Y Lee, John Q Trojanowski, Kurt R Brunden
A group of neurodegenerative diseases referred to as tauopathies are characterized by the presence of brain cells harboring inclusions of pathological species of the tau protein. These disorders include Alzheimer's disease and frontotemporal lobar degeneration due to tau pathology, including progressive supranuclear palsy, corticobasal degeneration, and Pick's disease. Tau is normally a microtubule (MT)-associated protein that appears to play an important role in ensuring proper axonal transport, but in tauopathies tau becomes hyperphosphorylated and disengages from MTs, with consequent misfolding and deposition into inclusions that mainly affect neurons but also glia...
October 2016: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
Stella Cecchetti, Zuzana Tatar, Pilar Galan, Bruno Pereira, Céline Lambert, Gael Mouterde, Angela Sutton, Martin Soubrier, Maxime Dougados
OBJECTIVES: The relationship between vitamin D and rheumatoid arthritis (RA) activity remains controversial. RA is a cardiovascular risk factor. A low level of vitamin D may increase blood pressure (BP) and decrease HDL-cholesterol. OBJECTIVES: We aimed to determine the prevalence of vitamin D deficiency in RA patients compared to controls, and also to investigate the relationship between vitamin D and RA activity, and between vitamin D and cardiovascular risk factors...
September 30, 2016: Clinical and Experimental Rheumatology
Sarah Jones, William D-C Man, Wei Gao, Irene J Higginson, Andrew Wilcock, Matthew Maddocks
BACKGROUND: This review is an update of a previously published review in the Cochrane Database of Systematic Reviews Issue 1, 2013 on Neuromuscular electrical stimulation for muscle weakness in adults with advanced disease.Patients with advanced progressive disease often experience muscle weakness, which can impact adversely on their ability to be independent and their quality of life. In those patients who are unable or unwilling to undertake whole-body exercise, neuromuscular electrical stimulation (NMES) may be an alternative treatment to enhance lower limb muscle strength...
October 17, 2016: Cochrane Database of Systematic Reviews
Alexandre N Rcom-H'cheo-Gauthier, Amelia Davis, Adrian C B Meedeniya, Dean L Pountney
α-Synuclein (α-syn) aggregates (Lewy bodies) in dementia with Lewy Bodies (DLB) may be associated with disturbed calcium homeostasis and oxidative stress. We investigated the interplay between α-syn aggregation, expression of the calbindin-D28k (CB) neuronal calcium-buffering protein and oxidative stress, combining immunofluorescence double labelling and Western analysis, and examining DLB and normal human cases and a unilateral oxidative stress lesion model of α-syn disease (rotenone mouse). DLB cases showed a greater proportion of CB+ cells in affected brain regions compared to normal cases with Lewy bodies largely present in CB- neurons and virtually undetected in CB+ neurons...
October 13, 2016: Molecular and Cellular Neurosciences
John S Bett
Cells have developed an evolutionary obligation to survey and maintain proteome fidelity and avoid the possible toxic consequences of protein misfolding and aggregation. Disturbances to protein homoeostasis (proteostasis) can result in severe cellular phenotypes and are closely linked with the accumulation of microscopically visible deposits of aggregated proteins. These include inclusion bodies found in AD (Alzheimer's disease), HD (Huntington's disease) and ALS (amyotrophic lateral sclerosis) patient neurons...
October 15, 2016: Essays in Biochemistry
Dhiman Ghosh, Surabhi Mehra, Shruti Sahay, Pradeep K Singh, Samir K Maji
Parkinson's disease (PD) is a neurological disorder marked by the presence of cytoplasmic inclusions, Lewy bodies (LBs) and Lewy neurites (LNs) as well as the degeneration of dopamine producing neurons in the substantia nigra region of the brain. The LBs and LNs in PD are mainly composed of aggregated form of a presynaptic protein, α-synuclein (α-Syn). However, the mechanisms of α-Syn aggregation and actual aggregated species responsible for the degeneration of dopaminergic neurons have not yet been resolved...
October 10, 2016: International Journal of Biological Macromolecules
Amalia E Yanni, Nikoleta S Stamataki, Panagiotis Konstantopoulos, Maria Stoupaki, Athanasios Abeliatis, Irene Nikolakea, Despoina Perrea, Vaios T Karathanos, Nikolaos Tentolouris
PURPOSE: Chromium (Cr)-enriched yeast supplementation to whole wheat bread (WWCrB) has been shown to ameliorate postprandial glycemic response in healthy subjects. The present study investigates the long-term benefit of WWCrB consumption for patients with type-2 diabetes mellitus (T2DM). METHODS: Thirty patients with T2DM were randomly assigned to a group receiving WWCrB or the plain whole wheat bread (WWB) group. Plasma glucose, insulin, glycosylated hemoglobin (HbA1c) and insulin resistance were determined, and oral glucose tolerance test (OGTT) was performed at the beginning and the end of the dietary intervention, which lasted for 12 weeks...
October 12, 2016: European Journal of Nutrition
Marco E Allaix, Edgar J B Furnée, Massimiliano Mistrangelo, Alberto Arezzo, Mario Morino
Laparoscopic resection for colon and rectal cancer is associated with quicker return of bowel function, reduced postoperative morbidity rates and shorter length of hospital stay compared to open surgery, with no differences in long-term survival. Conversion to open surgery is reported in up to 30% of patients enrolled in randomized control trials comparing open and laparoscopic colorectal resection for cancer. In this review, reasons for conversion are anatomical-related factors, disease-related-factors and surgeon-related factors...
October 7, 2016: World Journal of Gastroenterology: WJG
K McCloskey, A-L Ponsonby, F Collier, K Allen, M L K Tang, J B Carlin, R Saffery, M R Skilton, M Cheung, S Ranganathan, T Dwyer, D Burgner, P Vuillermin
BACKGROUND: Excess adiposity and adiposity-related inflammation are known risk factors for cardiovascular disease in adults; however, little is known regarding the determinants of adiposity-related inflammation at birth. OBJECTIVES: The aim of this study was to investigate the association between maternal pre-pregnancy BMI and newborn adiposity and inflammation. METHODS: Paired maternal (28-week gestation) and infant (umbilical cord) blood samples were collected from a population-derived birth cohort (Barwon Infant Study, n = 1074)...
October 9, 2016: Pediatric Obesity
Maninder Ahuja
INTRODUCTION: Age of menopause is a very important biomarker of not only the loss of fertility but also an increased risk for various mid-life diseases and problems. Many of these diseases can be prevented by timely intervention of lifestyle modification, menopausal hormone therapy, or other supplementations such as calcium, Vitamin D, and micronutrients. In India age of menopause is less than our counterparts in the Western world. This means that the fertility potential of Indian women starts compromising early, so we need to start with the preventive measures much early...
July 2016: Journal of Mid-life Health
Maria Asunción García Gonzalo, María Isabel Santamaría Mas, Lidia Pascual Tomé, Maialen Ibarguren Pinilla, Francisco Rodríguez-Arrondo
AIM: To assess the prevalence of comorbidities, concomitant therapies and adverse effects associated with the medication in a cohort of patients with HIV infection. DESIGN: Multicentre cross-sectional study. SETTINGS: Infectious Diseases or Internal Medicine outpatient Clinics of 3 hospitals in the Basque Country. PARTICIPANTS: During a 3 month period, patients with the following inclusion criteria were randomly selected: HIV infection, age>18years, antiretroviral treatment (ART) for at least 6months, and no changes in ART in the previous 4weeks...
October 5, 2016: Atencion Primaria
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