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https://www.readbyqxmd.com/read/28636584/proton-magnetic-resonance-spectroscopy-in-huntington-s-disease-accompanying-neuroborreliosis
#1
Helena Sarac, David Ozretic, Neven Henigsberg, Pero Hrabac, Ivan Bagaric, Lucija Bagaric-Krakan
No abstract text is available yet for this article.
June 2017: Psychiatria Danubina
https://www.readbyqxmd.com/read/28633181/agricultural-medicine-2nd-edition
#2
Mark K Huntington
No abstract text is available yet for this article.
June 2017: Family Medicine
https://www.readbyqxmd.com/read/28633139/mitochondrial-metabolism-in-a-large-animal-model-of-huntington-disease-the-hunt-for-biomarkers-in-the-spermatozoa-of-presymptomatic-minipigs
#3
Jana Krizova, Hana Stufkova, Marie Rodinova, Monika Macakova, Bozena Bohuslavova, Daniela Vidinska, Jiri Klima, Zdenka Ellederova, Antonin Pavlok, David S Howland, Jiri Zeman, Jan Motlik, Hana Hansikova
BACKGROUND: Huntington disease (HD) is a fatal neurodegenerative disorder involving reduced muscle coordination, mental and behavioral changes, and testicular degeneration. In order to further clarify the decreased fertility and penetration ability of the spermatozoa of transgenic HD minipig boars (TgHD), we applied a set of mitochondrial metabolism (MM) parameter measurements to this promising biological material, which can be collected noninvasively in longitudinal studies. OBJECTIVE: We aimed to optimize methods for MM measurements in spermatozoa and to establish possible biomarkers of HD in TgHD spermatozoa expressing the N-terminal part of mutated human huntingtin...
June 21, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28632780/metformin-intake-associates-with-better-cognitive-function-in-patients-with-huntington-s-disease
#4
David Hervás, Victoria Fornés-Ferrer, Ana Pilar Gómez-Escribano, María Dolores Sequedo, Carmen Peiró, José María Millán, Rafael P Vázquez-Manrique
Huntington's disease (HD) is an inherited, dominant neurodegenerative disorder caused by an abnormal expansion of CAG triplets in the huntingtin gene (htt). Despite extensive efforts to modify the progression of HD thus far only symptomatic treatment is available. Recent work suggests that treating invertebrate and mice HD models with metformin, a well-known AMPK activator which is used worldwide to treat type 2-diabetes, reduces mutant huntingtin from cells and alleviates many of the phenotypes associated to HD...
2017: PloS One
https://www.readbyqxmd.com/read/28632757/molecular-mechanism-of-drp1-assembly-studied-in-vitro-by-cryo-electron-microscopy
#5
Kaustuv Basu, Driss Lajoie, Tristan Aumentado-Armstrong, Jin Chen, Roman I Koning, Blaise Bossy, Mihnea Bostina, Attila Sik, Ella Bossy-Wetzel, Isabelle Rouiller
Mitochondria are dynamic organelles that continually adapt their morphology by fusion and fission events. An imbalance between fusion and fission has been linked to major neurodegenerative diseases, including Huntington's, Alzheimer's, and Parkinson's diseases. A member of the Dynamin superfamily, dynamin-related protein 1 (DRP1), a dynamin-related GTPase, is required for mitochondrial membrane fission. Self-assembly of DRP1 into oligomers in a GTP-dependent manner likely drives the division process. We show here that DRP1 self-assembles in two ways: i) in the presence of the non-hydrolysable GTP analog GMP-PNP into spiral-like structures of ~36 nm diameter; and ii) in the presence of GTP into rings composed of 13-18 monomers...
2017: PloS One
https://www.readbyqxmd.com/read/28632502/targeting-activated-protein-c-to-treat-hemophilia
#6
Stéphanie G I Polderdijk, Trevor P Baglin, James A Huntington
PURPOSE OF REVIEW: Hemophilia is a debilitating disease, marked by frequent, painful bleeding events, joint deterioration and early death. All current treatments consist of i.v. infusions of replacement factor or other procoagulant factors, and are incompletely effective, due in part to the short half-lives of the proteins. An alternative approach is to rebalance hemostasis by inhibiting natural anticoagulant mechanisms. In this article, we explain why activated protein C (APC) is an appropriate and safe target for the treatment of hemophilia...
June 16, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28632387/poly-trehalose-nanoparticle-prevents-amyloid-aggregation-and-suppress-polyglutamine-aggregation-in-huntington-s-disease-model-mouse
#7
Koushik Debnath, Nibedita Pradhan, Brijesh Kumar Singh, Nihar R Jana, Nikhil R Jana
Prevention and therapeutic strategy of various neurodegenerative disease focus on inhibiting protein fibrillation, clearing of aggregated protein plaques from brain and lowering of protein aggregate-induced toxicity. We have designed poly(trehalose) nanoparticle that can inhibit amyloid/polyglutamine aggregation under extra-/intra-cellular condition, reduce such aggregation-derived cytotoxicity and prevents polyglutamine aggregation in Huntington's disease (HD) model mouse brain. Nanoparticle has 20-30 nm hydrodynamic size, composed of 6 nm iron oxide core and zwitterionic polymer shell with covalently linked trehalose of ~5-12 wt %...
June 20, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28628549/a-classification-system-to-guide-physical-therapy-management-in-huntington-disease-a-case-series
#8
Nora E Fritz, Monica Busse, Karen Jones, Hanan Khalil, Lori Quinn
BACKGROUND AND PURPOSE: Individuals with Huntington disease (HD), a rare neurological disease, experience impairments in mobility and cognition throughout their disease course. The Medical Research Council framework provides a schema that can be applied to the development and evaluation of complex interventions, such as those provided by physical therapists. Treatment-based classifications, based on expert consensus and available literature, are helpful in guiding physical therapy management across the stages of HD...
July 2017: Journal of Neurologic Physical Therapy: JNPT
https://www.readbyqxmd.com/read/28628038/crispr-cas9-mediated-gene-editing-ameliorates-neurotoxicity-in-mouse-model-of-huntington-s-disease
#9
Su Yang, Renbao Chang, Huiming Yang, Ting Zhao, Yan Hong, Ha Eun Kong, Xiaobo Sun, Zhaohui Qin, Peng Jin, Shihua Li, Xiao-Jiang Li
Huntington's disease is a neurodegenerative disorder caused by a polyglutamine repeat in the Huntingtin gene (HTT). Although suppressing the expression of mutant HTT (mHTT) has been explored as a therapeutic strategy to treat Huntington's disease, considerable efforts have gone into developing allele-specific suppression of mHTT expression, given that loss of Htt in mice can lead to embryonic lethality. It remains unknown whether depletion of HTT in the adult brain, regardless of its allele, could be a safe therapy...
June 19, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28625786/drug-induces-depression-like-phenotypes-and-alters-gene-expression-profiles-in-drosophila
#10
Ming-Di Jiang, Ya Zheng, Jia-Lin Wang, Yu-Feng Wang
BACKGROUND: Major depressive disorder (MDD) is a severe mental illness that affects more than 350 million people worldwide. However, the molecular mechanisms of depression are currently unclear. Studies suggest that Drosophila and humans have similar depression-like symptoms under pressure. In this research, we choose Drosophila melanogaster as the animal model to explore the molecular mechanisms that trigger depression. RESULTS: We found that feeding D. melanogaster with the medium containing Levodopa or Chlorpromazine could induce depression-like phenotypes in both behavioral and biochemical biomarkers, including significantly decreased food intake, mating frequency, serotonin (5-HT) concentration, and increased malondialdehyde (MDA) concentration as well as reduced activity of superoxide dismutase (SOD)...
June 15, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28624355/3-np-induced-huntington-s-like-disease-impairs-nrf2-activation-without-loss-of-cardiac-function-in-aged-rats
#11
A Silva-Palacios, M Ostolga-Chavarría, M Buelna-Chontal, C Garibay, S Hernández-Reséndiz, F J Roldán, P L Flores, A Luna-López, M Königsberg, C Zazueta
Cardiovascular diseases (CVDs) are one of the leading causes of death in patients over 60years with Huntington's disease (HD). Here, we investigated if age-related oxidative stress (OS) is a relevant factor to develop cardiac damage in an in vivo model of striatal neurodegeneration induced by 3-nitropropionic acid (3-NP). We also evaluated the potential effect of tert-butylhydroquinone (tBHQ) to increase the Nrf2-regulated antioxidant response in hearts from adult and aged rats intoxicated with 3-NP. Our results showed that 3-NP-treatment did not induce cardiac dysfunction, neither in adult nor in aged rats...
June 14, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28624208/safe-and-efficient-silencing-with-a-pol-ii-but-not-a-pol-lii-promoter-expressing-an-artificial-mirna-targeting-human-huntingtin
#12
Edith L Pfister, Kathryn O Chase, Huaming Sun, Lori A Kennington, Faith Conroy, Emily Johnson, Rachael Miller, Florie Borel, Neil Aronin, Christian Mueller
Huntington's disease is a devastating, incurable neurodegenerative disease affecting up to 12 per 100,000 patients worldwide. The disease is caused by a mutation in the Huntingtin (Htt) gene. There is interest in reducing mutant Huntingtin by targeting it at the mRNA level, but the maximum tolerable dose and long-term effects of such a treatment are unknown. Using a self-complementary AAV9 vector, we delivered a mir-155-based artificial miRNA under the control of the chicken β-actin or human U6 promoter. In mouse brain, the artificial miRNA reduced the human huntingtin mRNA by 50%...
June 16, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28621841/hypomethylating-agent-therapy-use-and-survival-in-older-patients-with-chronic-myelomonocytic-leukemia-in-the-united-states-a-large-population-based-study
#13
Amer M Zeidan, Xin Hu, Jessica B Long, Rong Wang, Xiaomei Ma, Nikolai A Podoltsev, Scott F Huntington, Steven D Gore, Amy J Davidoff
BACKGROUND: Despite the approval of azacitidine in 2004 and the approval of decitabine in 2006 in the United States for chronic myelomonocytic leukemia (CMML), the overall survival (OS) benefit with hypomethylating agent (HMA) therapy is unclear. METHODS: Older adults (age ≥ 66 years) who had been diagnosed with CMML from 2001 to 2011 were selected from the Surveillance, Epidemiology, and End Results-Medicare database, and propensity score matching was used to match patients who had been diagnosed after HMA approval (2007-2011) and had received HMA treatment with patients diagnosed before HMA approval (2001-2003)...
June 16, 2017: Cancer
https://www.readbyqxmd.com/read/28621715/neuropsychiatric-burden-in-huntington-s-disease
#14
REVIEW
Ricardo Augusto Paoli, Andrea Botturi, Andrea Ciammola, Vincenzo Silani, Cecilia Prunas, Claudio Lucchiari, Elisa Zugno, Elisabetta Caletti
Huntington's disease is a disorder that results in motor, cognitive, and psychiatric problems. The symptoms often take different forms and the presence of disturbances of the psychic sphere reduces patients' autonomy and quality of life, also impacting patients' social life. It is estimated that a prevalence between 33% and 76% of the main psychiatric syndromes may arise in different phases of the disease, often in atypical form, even 20 years before the onset of chorea and dementia. We present a narrative review of the literature describing the main psychopathological patterns that may be found in Huntington's disease, searching for a related article in the main database sources (Medline, ISI Web of Knowledge, Scopus, and Medscape)...
June 16, 2017: Brain Sciences
https://www.readbyqxmd.com/read/28621522/formation-and-structure-of-wild-type-huntingtin-exon-1-fibrils
#15
J Mario Isas, Andreas Langen, Myles C Isas, Nitin Kumar Pandey, Ansgar B Siemer
The fact that the heritable neurodegenerative disorder Huntington's Disease (HD) is autosomal dominant means that there is one wild type and one mutant allele in most HD patients. The CAG repeat expansion in the exon 1 of the protein huntingtin (HTTex1) that causes the disease, leads to the formation of HTT fibrils in vitro and vivo. An important question for understanding the molecular mechanism of HD is which role wild type HTT plays for the formation, propagation, and structure of these HTT fibrils. Here we report that fibrils of mutant HTTex1 are able to seed the aggregation of wild type HTTex1 into amyloid fibrils which in turn can seed the fibril formation of mutant HTTex1...
June 16, 2017: Biochemistry
https://www.readbyqxmd.com/read/28617590/structure-and-dynamics-of-rna-repeat-expansions-that-cause-huntington-s-disease-and-myotonic-dystrophy-type-1
#16
Jonathan L Chen, Damian M VanEtten, Matthew A Fountain, Ilyas Yildirim, Matthew D Disney
RNA repeat expansions cause a host of incurable, genetically-defined diseases. The most common class of RNA repeats are trinucleotide repeats. These long, repeating transcripts fold into hairpins containing 1 × 1 internal loops that can mediate disease via a variety of mechanism(s) in which RNA is the central player. Two of these disorders are Huntington's disease and myotonic dystrophy type 1, which are caused by r(CAG) and r(CUG) repeats, respectively. We report the structures of two RNA constructs containing three copies of a r(CAG) [r(3×CAG)] or r(CUG) [r(3×CUG)] motif that were modeled with nuclear magnetic resonance spectroscopy and simulated annealing with restrained molecular dynamics...
June 15, 2017: Biochemistry
https://www.readbyqxmd.com/read/28615996/international-society-of-sports-nutrition-position-stand-safety-and-efficacy-of-creatine-supplementation-in-exercise-sport-and-medicine
#17
REVIEW
Richard B Kreider, Douglas S Kalman, Jose Antonio, Tim N Ziegenfuss, Robert Wildman, Rick Collins, Darren G Candow, Susan M Kleiner, Anthony L Almada, Hector L Lopez
Creatine is one of the most popular nutritional ergogenic aids for athletes. Studies have consistently shown that creatine supplementation increases intramuscular creatine concentrations which may help explain the observed improvements in high intensity exercise performance leading to greater training adaptations. In addition to athletic and exercise improvement, research has shown that creatine supplementation may enhance post-exercise recovery, injury prevention, thermoregulation, rehabilitation, and concussion and/or spinal cord neuroprotection...
2017: Journal of the International Society of Sports Nutrition
https://www.readbyqxmd.com/read/28613370/-delayed-diagnosis-of-huntington-s-disease-in-a-psychiatric-setting
#18
L M Tak, B Sizoo, B M Aan de Stegge, S Adema, E van Duijn, B Kremer
Huntington's disease (hd) is characterised by a triad of neuropsychiatric symptoms, motor disturbances and cognitive decline. If initial symptoms are of neuropsychiatric nature they maybe misinterpreted, which can lead to delayed diagnosis. Three examples of delayed hd diagnosis in a psychiatric setting are discussed.
2017: Tijdschrift Voor Psychiatrie
https://www.readbyqxmd.com/read/28611660/neurodegeneration-and-the-circadian-clock
#19
REVIEW
Suzanne Hood, Shimon Amir
Despite varied etiologies and symptoms, several neurodegenerative diseases-specifically, Alzheimer's (AD), Parkinson's (PD), and Huntington's diseases (HDs)-share the common feature of abnormal circadian rhythms, such as those in behavior (e.g., disrupted sleep/wake cycles), physiological processes (e.g., diminished hormone release) and biochemical activities (e.g., antioxidant production). Circadian disturbances are among the earliest symptoms of these diseases, and the molecular mechanisms of the circadian system are suspected to play a pivotal, and possibly causal, role in their natural histories...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28611656/dissecting-the-molecular-mechanisms-of-neurodegenerative-diseases-through-network-biology
#20
Jose A Santiago, Virginie Bottero, Judith A Potashkin
Neurodegenerative diseases are rarely caused by a mutation in a single gene but rather influenced by a combination of genetic, epigenetic and environmental factors. Emerging high-throughput technologies such as RNA sequencing have been instrumental in deciphering the molecular landscape of neurodegenerative diseases, however, the interpretation of such large amounts of data remains a challenge. Network biology has become a powerful platform to integrate multiple omics data to comprehensively explore the molecular networks in the context of health and disease...
2017: Frontiers in Aging Neuroscience
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