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Zoltan Ujfalusi, Carlos D Vera, Srbolujub M Mijailovich, Marina Svicevic, Elizabeth Choe Yu, Masataka Kawana, Kathleen Ruppel, James A Spudich, Michael A Geeves, Leslie A Leinwand
Dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM) can cause arrhythmias, heart failure, and cardiac death. Here, we functionally characterized the motor domains of five DCM-causing mutations in human β-cardiac myosin. Kinetic analyses of the individual events in the ATPase cycle revealed that each mutation alters different steps in this cycle. For example, different mutations gave enhanced or reduced rate constants of ATP binding, ATP hydrolysis, or ADP release or exhibited altered ATP, ADP, or actin affinity...
April 17, 2018: Journal of Biological Chemistry
E Dutton, J López-Alvarez
Dilated cardiomyopathy is the second most common cardiac disease in dogs and causes considerable morbidity and mortality. Primary dilated cardiomyopathy is suspected to be familial, and genetic loci have been associated with the disease in a number of breeds. Because it is an adult-onset disease, usually with late onset, testing breeding dogs and bitches before breeding for a genetic mutation that could lead to dilated cardiomyopathy would be helpful to prevent disease. There is growing evidence that the genetic basis may be multigenic rather than monogenic in the majority of studied breeds...
April 17, 2018: Journal of Small Animal Practice
Allan R Martin, Benjamin De Leener, Julien Cohen-Adad, Sukhvinder Kalsi-Ryan, David W Cadotte, Jefferson R Wilson, Lindsay Tetreault, Aria Nouri, Adrian Crawley, David J Mikulis, Howard Ginsberg, Eric M Massicotte, Michael G Fehlings
BACKGROUND: Patients with mild degenerative cervical myelopathy (DCM) are often managed non-operatively, and surgery is recommended if neurological progression occurs. However, detection of progression is often subjective. Quantitative MRI (qMRI) directly measures spinal cord (SC) tissue changes, detecting axonal injury, demyelination, and atrophy. This longitudinal study compared multiparametric qMRI with clinical measures of progression in non-operative DCM patients. METHODS: 26 DCM patients were followed...
2018: PloS One
Abdul Badran, Benjamin M Davies, Heidi-Marie Bailey, Sukhvinder Kalsi-Ryan, Mark Rn Kotter
OBJECTIVE: To review peer-reviewed literature relating to postoperative physiotherapy for degenerative cervical myelopathy (DCM), to determine efficacy in improving clinical outcome and recovery. DATA SOURCES: MEDLINE, EMBASE, CENTRAL, PEDro, ISRCTN registry, WHO ICTRP and . References and citations of relevant articles were searched. METHODS: A systematic search was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (PROSPERO CRD42016039511) from the origins of the databases till 15 February 2018...
April 1, 2018: Clinical Rehabilitation
Haibin Xiao, Chuanchen Wu, Ping Li, Bo Tang
Diabetic cardiomyopathy (DCM) is a critical complication of diabetes, the accurate pathogenesis of which remains elusive. It's widely accepted that endoplasmic reticulum (ER) stress and abnormal fluctuations of reactive oxygen species (ROS) are considered to be closely associated with progress of DCM. In addition, DCM-induced changes of myocardial tissue and ROS-derived oxidation of proteins will cause changes of the hydrophilic and hydrophobic domains and may further seriously alter the myocardial cell polarity...
April 17, 2018: Analytical Chemistry
Stefano Pedretti, Sara Vargiu, Matteo Baroni, Santo Dellegrottaglie, Barbara Lanzarin, Alberto Roghi, Angela Milazzo, Giuseppina Quattrocchi, Maurizio Lunati, Patrizia Pedrotti
BACKGROUND: Late gadolinium enhancement (LGE) assessed with cardiovascular magnetic resonance (CMR) correlates with ventricular arrhythmias and survival in patients with structural heart disease. Whether some LGE characteristics may specifically improve prediction of arrhythmic outcomes is unknown. HYPOTHESIS: We sought to evaluate scar characteristics assessed with CMR to predict implantable cardioverter-defibrillator (ICD) interventions in dilated cardiomyopathy of different etiology...
April 17, 2018: Clinical Cardiology
Fei Tong, Rongkui Chai, Haiying Jiang, Bo Dong
Background: The objective of this study was to survey the therapeutic function of curcumin-encapsulated poly(gamma-benzyl l-glutamate)-poly(ethylene glycol)-poly(gammabenzyl l-glutamate) (PBLG-PEG-PBLG) (P) on diabetic cardiomyopathy (DCM) via cross regulation effect of calcium-sensing receptor (CaSR) and endogenous cystathionine-γ-lyase (CSE)/hydrogen sulfide (H2 S). Methods: Diabetic rats were preconditioned with 20 mg/kg curcumin or curcumin/P complex continuously for 8 weeks...
2018: International Journal of Nanomedicine
Suma Uday, Wolfgang Högler
The consequences of vitamin D and dietary calcium deficiency have become a huge public health concern in the UK. The burden of disease from these deficiencies includes rickets, and hypocalcaemic seizures, dilated cardiomyopathy and mostly occult myopathy and osteomalacia. The increasing burden of the disease is intrinsically linked to ethnicity and the population demographic changes in the UK. Three facts have led to the resurfacing of the English disease: (1) the UK has no ultraviolet sunlight for at least 6 months of the year, (2) dark skin produces far less vitamin D than white skin per unit ultraviolet light exposure, and (3) non-European Union immigration over the last century...
April 16, 2018: Archives of Disease in Childhood
Evan D Adams, Nicholas J Jackson, Tim Young, Eugene C DePasquale, Leigh C Reardon
BACKGROUND: MELD-XI (Model for End-Stage Liver Disease eXcluding INR) at cardiac transplant has demonstrated prognostic survival utility, but has not been specifically validated in adult congenital heart disease (ACHD) in a registry study. METHODS: Adults undergoing first time orthotopic heart transplant from 2005-2015 in the UNOS (United Network for Organ Sharing) registry were examined in parallel: ACHD (n=543), ischemic dilated cardiomyopathy (IDCM, n=6,954) and valvular heart disease (VHD, n=355)...
April 16, 2018: Clinical Transplantation
Golnaz Vaseghi, Mohsen Sharifi, Nasim Dana, Ahmad Ghasemi, Afsaneh Yegdaneh
Background: Marine organisms produce a variety of compounds with pharmacological activities including anticancer effects. This study attempt to find cytotoxicity of hexane (HEX), dichloromethane (DCM), and butanol (BUTOH) partitions of Sargassum angustifolium . Materials and Methods: S. angustifolium was collected from Bushehr, a Southwest coastline of Persian Gulf. The plant was extracted by maceration with methanol-ethyl acetate. The extract was evaporated under vacuum and partitioned by Kupchan method to yield HEX, DCM, and BUTOH partitions...
2018: Advanced Biomedical Research
Andreas Martinsson, Petter Oest, Maj-Britt Wiborg, Öyvind Reitan, J Gustav Smith
OBJECTIVES: Left ventricular ejection fraction (EF) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) are important surrogate markers of cardiac function and wall stress. Randomized trials of heart failure (HF) have shown improvements in survival in patients with reduced EF (<40%, HFrEF) but not with preserved EF (≥50%, HFpEF) or mid-range EF (40-49%, HFmrEF). Limited information is available on the trajectory of EF in contemporary heart failure management programs (HFMPs)...
April 14, 2018: Scandinavian Cardiovascular Journal: SCJ
Chiara Bulgarelli, Anna Blasi, Simon Arridge, Samuel Powell, Carina de Klerk, Victoria Southgate, Sabrina Brigadoi, William Penny, Sungho Tak, Antonia Hamilton
Tracking the connectivity of the developing brain from infancy through childhood is an area of increasing research interest, and fNIRS provides an ideal method for studying the infant brain as it is compact, safe and robust to motion. However, data analysis methods for fNIRS are still underdeveloped compared to those available for fMRI. Dynamic causal modelling (DCM) is an advanced connectivity technique developed for fMRI data, that aims to estimate the coupling between brain regions and how this might be modulated by changes in experimental conditions...
April 12, 2018: NeuroImage
Xiaohui Dai, Ying Peng, Bin Zhou, Chunmei Li, Huizi Song, Qingyu Dou, Xiaochuan Xie, Li Rao
OBJECTIVE: To assess the association of polymorphisms of oncostatin M receptor (OSMR) gene with dilated cardiomyopathy (DCM) in a Han Chinese population. METHODS: For 351 DCM patients and 418 healthy controls, two single nucleotide polymorphisms (SNPs) of the OSMR gene, namely rs2292016 (promoter, -100G/T) and rs2278329 (missense, Asp553Asn), were genotyped with a TaqMan SNP genotyping assay. Two hundred of the patients were also followed up for (49.85 ± 22.52) months...
April 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Jetan H Badhiwala, Christopher D Witiw, Farshad Nassiri, Muhammad A Akbar, Blessing Jaja, Jefferson R Wilson, Michael G Fehlings
STUDY DESIGN: Post-hoc analysis of 606 patients enrolled in the AOSpine CSM-NA or CSM-I prospective, multi-center cohort studies. OBJECTIVE: To determine the minimum clinically important difference (MCID) in SF-36v2 Physical Component Summary (PCS) and Mental Component Summary (MSC) scores in patients undergoing surgery for degenerative cervical myelopathy (DCM). SUMMARY OF BACKGROUND DATA: There has been a shift toward focus on patient reported outcomes (PROs) in spine surgery...
April 12, 2018: Spine
Divya Suthar, Debra A Dodd, Justin Godown
There is often a diagnostic dilemma in pediatric patients presenting with depressed ventricular function, as myocarditis and dilated cardiomyopathy (DCM) of other etiologies can appear very similar. Accurate identification is critical to guide treatment and to provide families with the most accurate expectation of long-term outcomes. The objective of this study was to identify patterns of clinical presentation and to assess non-invasive measures to differentiate patients with acute myocarditis from other forms of DCM...
April 12, 2018: Pediatric Cardiology
Maarten M G van den Hoogenhof, Abdelaziz Beqqali, Ahmad S Amin, Ingeborg van der Made, Simona Aufiero, Mohsin A F Khan, Cees A Schumacher, Joeri A Jansweijer, Karin Y van Spaendonck-Zwarts, Carol Ann Remme, Johannes Backs, Arie O Verkerk, Antonius Baartscheer, Yigal M Pinto, Esther E Creemers
Background -Mutations in RBM20 cause a clinically aggressive form of dilated cardiomyopathy (DCM), with an increased risk of malignant ventricular arrhythmias. RBM20 is a splicing factor that targets multiple pivotal cardiac genes, such as Titin (TTN) and Calcium/calmodulin-dependent kinase II delta (CAMK2D). Aberrant TTN splicing is thought to be the main determinant of RBM20-induced DCM, but is not likely to explain the increased risk of arrhythmias. Here, we investigated the extent at which RBM20 mutation carriers have an increased risk of arrhythmias and explore the underlying molecular mechanism...
April 12, 2018: Circulation
Muhammed Fatih Bayraktar, Ozcan Ozeke
BACKGROUND: The sleeping position plays an important role in overall health in both healthy individuals and heart failure (HF) patients, which complain of increasing dyspnea when adopting left lateral decubitus position (LLDP) that improves when turning over to the right lateral decubitus position (RLDP). Several theories have been proposed to explain this preference of HF patients; however, the underlying mechanisms remain unclear. METHOD: We evaluated consecutive dilated cardiomyopathy (DCMP) patients with regard to whether they had a sleeping position preference and analyzed early and late left ventricular filling velocities, tissue Doppler recordings, tricuspid annular plane systolic excursion (TAPSE), and left ventricular outflow tract time-velocity integral (LVOT-TVI)...
April 12, 2018: Echocardiography
Hiroshi Kawakami, Akiyoshi Ogimoto, Naohito Tokunaga, Kazuhisa Nishimura, Hideo Kawakami, Haruhiko Higashi, Chiharuko Iio, Tamami Kono, Jun Aono, Teruyoshi Uetani, Takayuki Nagai, Katsuji Inoue, Jun Suzuki, Shuntaro Ikeda, Takafumi Okura, Yasumasa Ohyagi, Yasuharu Tabara, Jitsuo Higaki
The cardiac phenotype of laminopathies is characterized by cardiac conduction disorders (CCDs) and dilated cardiomyopathy (DCM). Although laminopathies have been considered monogenic, they exhibit a remarkable degree of clinical variability. This case series aimed to detect the causal mutation and to investigate the causes of clinical variability in a Japanese family with inherited CCD and DCM.Of the five family members investigated, four had either CCD/DCM or CCD alone, while one subject had no cardiovascular disease and acted as a normal control...
April 6, 2018: International Heart Journal
Mustafa Kılıç, Ahmet Cevdet Ceylan, Utku Arman Örün, Esra Kılıç
Hypotonia-cystinuria syndrome is a very rare autosomal recessive contiguous gene deletion syndrome of PREPL and SLC3A1 at 2p21 with neuromuscular and neuroendocrinologic presentation. We report a two-year-six-month-old affected female infant and her five-month-old affected brother with a novel homozygous deletion in SLC3A1 and PREPL gene. Both of siblings had mild facial dysmorphism, hypotonia, feeding problems, failure to thrive, developmental delay. She also had dilated cardiomyopathy which differ from other reported patients...
April 7, 2018: Metabolic Brain Disease
Gerrie P Farman, Michael J Rynkiewicz, Marek Orzechowski, William Lehman, Jeffrey R Moore
Calcium regulation of cardiac muscle contraction is controlled by the thin-filament proteins troponin and tropomyosin bound to actin. In the absence of calcium, troponin-tropomyosin inhibits myosin-interactions on actin and induces muscle relaxation, whereas the addition of calcium relieves the inhibitory constraint to initiate contraction. Many mutations in thin filament proteins linked to cardiomyopathy appear to disrupt this regulatory switching. Here, we tested perturbations caused by mutant tropomyosins (E40K, DCM; and E62Q, HCM) on intra-filament interactions affecting acto-myosin interactions including those induced further by myosin association...
April 4, 2018: Archives of Biochemistry and Biophysics
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