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Inclusion body disease

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https://www.readbyqxmd.com/read/28333717/effect-of-resistance-exercise-on-muscle-metabolism-and-autophagy-in-sibm
#1
Jae-Hoon Jeong, Dae-Seung Yang, Dong-Ju Hwang, Joon-Yong Cho, Eun-Bum Kang
PURPOSE: Sporadic inclusion body myositis (sIBM), a muscular degenerative disease in the elderly, is an inflammatory myopathy characterized by muscle weakness in the wrist flexor, quadriceps, and tibialis anterior muscles. We aimed to identify the therapeutic effect of resistance exercise (RE) in improving sIBM symptoms in an sIBM animal model. METHODS: Six-week-old male Wistar rats were divided into a sham group (sham, n = 12), chloroquine-control group (CQ-con, n = 12), and chloroquine-RE group (CQ-RE, n = 12)...
March 23, 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/28329057/coronary-artery-calcification-and-risk-of-cardiovascular-disease-and-death-among-patients-with-chronic-kidney-disease
#2
Jing Chen, Matthew J Budoff, Muredach P Reilly, Wei Yang, Sylvia E Rosas, Mahboob Rahman, Xiaoming Zhang, Jason A Roy, Eva Lustigova, Lisa Nessel, Virginia Ford, Dominic Raj, Anna C Porter, Elsayed Z Soliman, Jackson T Wright, Myles Wolf, Jiang He
Importance: Coronary artery calcification (CAC) is highly prevalent in dialysis-naive patients with chronic kidney disease (CKD). However, there are sparse data on the association of CAC with subsequent risk of cardiovascular disease and all-cause mortality in this population. Objective: To study the prospective association of CAC with risk of cardiovascular disease and all-cause mortality among dialysis-naive patients with CKD. Design, Setting, and Participants: The prospective Chronic Renal Insufficiency Cohort study recruited adults with an estimated glomerular filtration rate of 20 to 70 mL/min/1...
March 22, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28323167/high-hydrostatic-pressure-enables-almost-100-refolding-of-recombinant-human-ciliary-neurotrophic-factor-from-inclusion-bodies-at-high-concentration
#3
Qi Wang, Yongdong Liu, Chun Zhang, Fangxia Guo, Cui Feng, Xiunan Li, Hong Shi, Zhiguo Su
Protein refolding from inclusion bodies (IBs) often encounters a problem of low recovery at high protein concentration. In this study, we demonstrated that high hydrostatic pressure (HHP) could simultaneously achieve high refolding concentration and high refolding yield for IBs of recombinant human ciliary neurotrophic factor (rhCNTF), a potential therapeutic for neurodegenerative diseases. The use of dilution refolding obtained 18% recovery at 3 mg/mL, even in the presence of 4 M urea. In contrast, HHP refolding could efficiently increase the recovery up to almost 100% even at 4 mg/mL...
March 18, 2017: Protein Expression and Purification
https://www.readbyqxmd.com/read/28322724/valosin-containing-protein-vcp-p97-inhibitors-relieve-mitofusin-dependent-mitochondrial-defects-due-to-vcp-disease-mutants
#4
Ting Zhang, Prashant Mishra, Bruce A Hay, David Chan, Ming Guo
Missense mutations of valosin-containing protein (VCP) cause an autosomal dominant disease known as inclusion body myopathy, Paget disease with frontotemporal dementia (IBMPFD) and other neurodegenerative disorders. The pathological mechanism of IBMPFD is not clear and there is no treatment. We show that endogenous VCP negatively regulates Mitofusin, which is required for outer mitochondrial membrane fusion. Because 90% of IBMPFD patients have myopathy, we generated an in vivo IBMPFD model in adult Drosophila muscle, which recapitulates disease pathologies...
March 21, 2017: ELife
https://www.readbyqxmd.com/read/28321169/inflammatory-bowel-disease-an-evaluation-of-health-information-on-the-internet
#5
Samy A Azer, Thekra I AlOlayan, Malak A AlGhamdi, Malak A AlSanea
AIM: To evaluate the quality and accuracy of websites written to the public on inflammatory bowel disease (IBD) (Crohn's disease and ulcerative colitis) and assess their readability level. METHODS: Google™, Bing™, and Yahoo™ search engines were searched independently by three researchers in December 2014. Only English-language websites were selected on the basis of predetermined inclusion and exclusion criteria. Researchers independently evaluated the quality of each website by using the DISCERN and the HONcode instruments...
March 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28320381/variation-in-readmission-and-mortality-following-hospitalisation-with-a-diagnosis-of-heart-failure-prospective-cohort-study-using-linked-data
#6
Rosemary J Korda, Wei Du, Cathy Day, Karen Page, Peter S Macdonald, Emily Banks
BACKGROUND: Hospitalisation for heart failure is common and post-discharge outcomes, including readmission and mortality, are often poor and are poorly understood. The purpose of this study was to examine patient- and hospital-level variation in the risk of 30-day unplanned readmission and mortality following discharge from hospital with a diagnosis of heart failure. METHODS: Prospective cohort study using data from the Sax Institute's 45 and Up Study, linking baseline survey (Jan 2006-April 2009) to hospital and mortality data (to Dec 2011)...
March 21, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/28320207/examination-of-cardiovascular-risk-factors-and-rurality-in-appalachian-children
#7
Christa L Lilly, Amna Umer, Lesley Cottrell, Lee Pyles, William Neal
INTRODUCTOIN: The prevalence of childhood cardiovascular disease (CVD) risk factors often increases in more rural geographic regions in the USA. However, research on the topic often has conflicting results. Researchers note differences in definitions of rurality and other factors that would lead to differences in inference, including appropriate use of statistical clustering analysis, representative data, and inclusion of individual-level covariates. The present study's objective was to examine CVD risk factors during childhood by geographic distribution in the US Appalachian region as a first step towards understanding the health disparities in this area...
January 2017: Rural and Remote Health
https://www.readbyqxmd.com/read/28318044/pathomechanisms-of-anti-cn1a-autoantibodies-in-sporadic-inclusion-body-myositis
#8
Nozomu Tawara, Satoshi Yamashita, Xiao Zhang, Mai Korogi, Ziwei Zhang, Tsukasa Doki, Yoshimasa Matsuo, Shunya Nakane, Yasushi Maeda, Kazuma Sugie, Naoki Suzuki, Masashi Aoki, Yukio Ando
OBJECTIVE: Sporadic inclusion body myositis (sIBM), an intractable progressive muscle disease, frequently occurs in older persons. sIBM pathogenesis may involve protein degradation dysfunction and immune abnormalities. Autoantibodies recognizing cytosolic 5'-nucleotidase 1A (cN1A) were found in plasma and serum from sIBM patients. Whether anti-cN1A autoantibodies play a pathogenic role in sIBM is controversial, however. This study investigated the pathogenic properties of anti-cN1A autoantibodies in sIBM pathogenesis...
March 20, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28316031/transactivation-domain-of-human-c-myc-is-essential-to-alleviate-poly-q-mediated-neurotoxicity-in-drosophila-disease-models
#9
Kritika Raj, Surajit Sarkar
Polyglutamine (poly(Q)) disorders, such as Huntington's disease (HD) and spinocerebellar ataxias, represent a group of neurological disorders which arise due to an atypically expanded poly(Q) tract in the coding region of the affected gene. Pathogenesis of these disorders inside the cells begins with the assembly of these mutant proteins in the form of insoluble inclusion bodies (IBs), which progressively sequester several vital cellular transcription factors and other essential proteins, and finally leads to neuronal dysfunction and apoptosis...
March 18, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28315306/molecular-characterization-of-camelpox-virus-isolates-from-bikaner-india-evidence-of-its-endemicity
#10
Shyam Singh Dahiya, Sachin Kumar, Sharat Chandra Mehta, Raghvendar Singh, Kashi Nath, Shirish D Narnaware, Fateh Chand Tuteja
Camelpox is an important viral disease of camels, which may produce mild skin lesions or severe systemic infections. Camelpox virus (CMLV) isolates retrieved from an incidence of camelpox in camels at Bikaner, India were characterized on the basis of genotype and pathotype. Histopathological examination of the CMLV scab revealed intracytoplasmic-eosinophilic inclusion bodies. The phylogenetic analysis of all eight CMLV isolates for C18L gene nucleotide sequence revealed its clustering with its strains M-96 from Kazakhstan and CMS from Iran...
March 14, 2017: Acta Tropica
https://www.readbyqxmd.com/read/28306532/evaluation-of-the-sub-chronic-toxicity-profile-of-the-corm-of-xanthosoma-sagittifolium-on-hematology-and-biochemistry-of-alloxan-induced-diabetic-wistar-rats
#11
Olayinka A Oridupa, Oluyemisi F Folasire, Adedotun J Owolabi
Background Hematological and biochemical changes associated with diabetes mellitus and probable reversal were assessed in alloxan-induced diabetic Wistar rats fed with varied percentages of Xanthosoma sagittifolium corm feed (Xs). The changes were compared to normoglycemic rats and diabetic rats treated with glibenclamide. Methods The study had eight groups in all with group 8 (control) consisting of five normoglycemic rats fed with normal rat pellets (Nrp). Diabetes was experimentally induced by intraperitoneal injection of alloxan to normoglycemic rats...
March 11, 2017: Journal of Complementary & Integrative Medicine
https://www.readbyqxmd.com/read/28303975/a-conserved-inter-domain-communication-mechanism-regulates-the-atpase-activity-of-the-aaa-protein-drg1
#12
Michael Prattes, Mathias Loibl, Gertrude Zisser, Daniel Luschnig, Lisa Kappel, Ingrid Rössler, Manuela Grassegger, Altijana Hromic, Elmar Krieger, Karl Gruber, Brigitte Pertschy, Helmut Bergler
AAA-ATPases fulfil essential roles in different cellular pathways and often act in form of hexameric complexes. Interaction with pathway-specific substrate and adaptor proteins recruits them to their targets and modulates their catalytic activity. This substrate dependent regulation of ATP hydrolysis in the AAA-domains is mediated by a non-catalytic N-terminal domain. The exact mechanisms that transmit the signal from the N-domain and coordinate the individual AAA-domains in the hexameric complex are still the topic of intensive research...
March 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28300069/extracellular-%C3%AE-synuclein-induces-sphingosine-1-phosphate-receptor-subtype-1-uncoupled-from-inhibitory-g-protein-leaving-%C3%AE-arrestin-signal-intact
#13
Lifang Zhang, Taro Okada, Shaymaa Mohamed Mohamed Badawy, Chihoko Hirai, Taketoshi Kajimoto, Shun-Ichi Nakamura
Parkinson's disease (PD) is the second most common neurodegenerative disorder. The presence of α-synuclein (α-Syn)-positive intracytoplasmic inclusions, known as Lewy bodies, is the cytopathological hallmark of PD. Increasing bodies of evidence suggest that cell-to-cell transmission of α-Syn plays a role in the progression of PD. Although extracellular α-Syn is known to cause abnormal cell motility, the precise mechanism remains elusive. Here we show that impairment of platelet-derived growth factor-induced cell motility caused by extracellular α-Syn is mainly attributed to selective inhibition of sphingosine 1-phosphate (S1P) signalling...
March 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28296815/anterior-trunk-mobility-does-not-predict-disability-in-elderly-women-with-acute-low-back-pain-brazilian-back-complaints-in-the-elders-bace-brazil-study-results
#14
Diogo Carvalho Felicio, Daniele Sirineu Pereira, Juliano Bergamaschine Mata Diz, Barbara Zille de Queiroz, Juscelio Pereira da Silva, Amanda Aparecida Oliveira Leopoldino, Leani Souza Maximo Pereira
STUDY DESIGN: Cross-sectional, ancillary study of an international multicenter epidemiological study. OBJECTIVE: To investigate the relationship of the anterior trunk mobility with self-report and physical performance measures in elderly women with acute low back pain (LBP). SUMMARY OF BACKGROUND DATA: LBP is one of the most prevalent pain complaints in the elderly population. It is postulated that the increased range of motion (ROM) of limited joints of the trunk may improve LBP and functionality of patients...
March 14, 2017: Spine
https://www.readbyqxmd.com/read/28296376/-lafora-disease-presentation-two-cases-in-a-mexican-family
#15
María Guadalupe González-De la Rosa, Edith Alva-Moncayo
Myoclonic epilepsy, described in 1911 by Lafora and Glueck, is an autosomal recessive hereditary clinical-pathological entity, which begins at the end of childhood or during adolescence, presents atypical absences, generalized and atonic tonic-clonic seizures, which can evolve to the epileptic state. The diagnosis is confirmed trough the skin biopsy or trough determination of the protein laforine. In this paper we present the initial case of a patient in whom we confirm the diagnosis of progressive myoclonic epilepsy and in particular the Lafora disease, which due to the symptomatology and the knowledge of the case we were able to detect her sister's disease...
March 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28295916/trehalose-activates-autophagy-and-decreases-proteasome-inhibitor-induced-endoplasmic-reticulum-stress-and-oxidative-stress-mediated-cytotoxicity-in-hepatocytes
#16
Yuichi Honma, Miyuki Sato-Morita, Yuka Katsuki, Hitomi Mihara, Ryoko Baba, Masaru Harada
Endoplasmic reticulum stress is associated with the pathophysiology of various liver diseases. Endoplasmic reticulum stress mediates the accumulation of abnormal proteins and leads to oxidative stress, cytoplasmic inclusion body formation and apoptosis in hepatocytes. Autophagy is a bulk degradation pathway for long-lived cytoplasmic proteins or damaged organelles and is also a major degradation pathway for many aggregate-prone and disease-causing proteins. We previously reported that rapamycin, a mammalian target of rapamycin inhibitor, activated autophagy and decreased proteasome inhibitor-mediated ubiquitinated protein accumulation, cytoplasmic inclusion body formation and apoptosis in hepatocytes...
March 11, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28295599/gender-difference-in-the-association-and-presentation-of-visual-hallucinations-in-dementia-with-lewy-bodies-a-cross-sectional-study
#17
Pai-Yi Chiu, Po-Ren Teng, Cheng-Yu Wei, Chein-Wei Wang, Chun-Tang Tsai
OBJECTIVE: Visual hallucinations (VHs) are among the most striking features of dementia with Lewy bodies (DLB). We investigated gender differences in the association and presentation of VHs in DLB. METHODS: A cross-sectional analysis of baseline data from a prospective, longitudinal study on dementia was performed. Cumulative frequency, 1-month frequency, and phenomenology of VHs were summarized and compared between female and male patients with DLB. Gender differences in the factors associated with VHs were investigated in patients with and without hallucinations...
March 13, 2017: International Journal of Geriatric Psychiatry
https://www.readbyqxmd.com/read/28292782/exploring-the-fitness-hypothesis-in-als-a-population-based-case-control-study-of-parental-cause-of-death-and-lifespan
#18
Anne E Visser, Meinie Seelen, Alexander Hulsbergen, Joris de Graaf, Anneke J van der Kooi, Joost Raaphorst, Jan H Veldink, Leonard H van den Berg
OBJECTIVE: To investigate the theory of premorbid fitness in amyotrophic lateral sclerosis (ALS), we studied whether a common genetic profile for physical or cardiovascular fitness was manifest in progenitors leading to less cardiovascular death and a longer lifespan in parents of patients with ALS compared with parents of controls. METHODS: Patient and disease characteristics, levels of physical activity, parental cause and age of death were obtained using a structured questionnaire from a population-based, case-control study of ALS in the Netherlands...
March 14, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28288686/outcomes-of-dysvascular-partial-foot-amputation-and-how-these-compare-to-transtibial-amputation-a-systematic-review-for-the-development-of-shared-decision-making-resources
#19
Michael P Dillon, Matthew Quigley, Stefania Fatone
BACKGROUND: Dysvascular partial foot amputation (PFA) is a common sequel to advanced peripheral vascular disease. Helping inform difficult discussions between patients and practitioners about the level of PFA, or the decision to have a transtibial amputation (TTA) as an alternative, requires an understanding of the current research evidence on a wide range of topics including wound healing, reamputation, quality of life, mobility, functional ability, participation, pain and psychosocial outcomes, and mortality...
March 14, 2017: Systematic Reviews
https://www.readbyqxmd.com/read/28281308/overlapping-but-distinct-tdp-43-and-tau-pathologic-patterns-in-aged-hippocampi
#20
Vanessa D Smith, Adam D Bachstetter, Eseosa Ighodaro, Kelly Roberts, Erin L Abner, David W Fardo, Peter T Nelson
Intracellular proteinaceous aggregates (inclusion bodies) are almost always detectable at autopsy in brains of elderly individuals. Inclusion bodies composed of TDP-43 and tau proteins often coexist in the same brain, and each of these pathologic biomarkers is associated independently with cognitive impairment. However, uncertainties remain about how the presence and neuroanatomical distribution of inclusion bodies correlate with underlying diseases including Alzheimer's disease (AD). To address this knowledge gap, we analyzed data from the University of Kentucky AD Center autopsy series (n = 247); none of the brains had frontotemporal lobar degeneration...
March 9, 2017: Brain Pathology
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