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https://www.readbyqxmd.com/read/27911442/palmitoylation-of-caspase-6-by-hip14-regulates-its-activation
#1
Niels H Skotte, Shaun S Sanders, Roshni R Singaraja, Dagmar E Ehrnhoefer, Kuljeet Vaid, Xiaofan Qiu, Srinivasaragavan Kannan, Chandra Verma, Michael R Hayden
Caspase-6 (CASP6) has an important role in axonal degeneration during neuronal apoptosis and in the neurodegenerative diseases Alzheimer and Huntington disease. Decreasing CASP6 activity may help to restore neuronal function in these and other diseases such as stroke and ischemia, where increased CASP6 activity has been implicated. The key to finding approaches to decrease CASP6 activity is a deeper understanding of the mechanisms regulating CASP6 activation. We show that CASP6 is posttranslationally palmitoylated by the palmitoyl acyltransferase HIP14 and that the palmitoylation of CASP6 inhibits its activation...
December 2, 2016: Cell Death and Differentiation
https://www.readbyqxmd.com/read/27910586/a-novel-method-to-recover-dd-fusion-proton-cr-39-data-corrupted-by-fast-ablator-ions-at-omega-and-the-national-ignition-facility
#2
G D Sutcliffe, L M Milanese, D Orozco, B Lahmann, M Gatu Johnson, F H Séguin, H Sio, J A Frenje, C K Li, R D Petrasso, H-S Park, J R Rygg, D T Casey, R Bionta, D P Turnbull, C M Huntington, J S Ross, A B Zylstra, M J Rosenberg, V Yu Glebov
CR-39 detectors are used routinely in inertial confinement fusion (ICF) experiments as a part of nuclear diagnostics. CR-39 is filtered to stop fast ablator ions which have been accelerated from an ICF implosion due to electric fields caused by laser-plasma interactions. In some experiments, the filtering is insufficient to block these ions and the fusion-product signal tracks are lost in the large background of accelerated ion tracks. A technique for recovering signal in these scenarios has been developed, tested, and implemented successfully...
November 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27910515/proton-pinhole-imaging-on-the-national-ignition-facility
#3
A B Zylstra, H-S Park, J S Ross, F Fiuza, J A Frenje, D P Higginson, C Huntington, C K Li, R D Petrasso, B Pollock, B Remington, H G Rinderknecht, D Ryutov, F H Séguin, D Turnbull, S C Wilks
Pinhole imaging of large (mm scale) carbon-deuterium (CD) plasmas by proton self-emission has been used for the first time to study the microphysics of shock formation, which is of astrophysical relevance. The 3 MeV deuterium-deuterium (DD) fusion proton self-emission from these plasmas is imaged using a novel pinhole imaging system, with up to five different 1 mm diameter pinholes positioned 25 cm from target-chamber center. CR39 is used as the detector medium, positioned at 100 cm distance from the pinhole for a magnification of 4 ×...
November 2016: Review of Scientific Instruments
https://www.readbyqxmd.com/read/27908981/neurotrophin-signalling-novel-insights-into-mechanisms-and-pathophysiology
#4
REVIEW
Mariela Mitre, Abigail Mariga, Moses V Chao
Neurotrophins, such as brain-derived neurotrophic factor (BDNF), are prominent regulators of neuronal survival, growth and differentiation during development. While trophic factors are viewed as well-understood but not innovative molecules, there are many lines of evidence indicating that BDNF plays an important role in the pathophysiology of many neurodegenerative disorders, depression, anxiety and other psychiatric disorders. In particular, lower levels of BDNF are associated with the aetiology of Alzheimer's and Huntington's diseases...
January 1, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/27908782/adaptive-responses-of-neuronal-mitochondria-to-bioenergetic-challenges-roles-in-neuroplasticity-and-disease-resistance
#5
REVIEW
Sophia M Raefsky, Mark P Mattson
An important concept in neurobiology is "neurons that fire together, wire together" which means that the formation and maintenance of synapses is promoted by activation of those synapses. Very similar to the effects of the stress of exercise on muscle cells, emerging findings suggest that neurons respond to activity by activating signaling pathways (e.g., Ca(2+), CREB, PGC-1α, NF-κB) that stimulate mitochondrial biogenesis and cellular stress resistance. These pathways are also activated by aerobic exercise and food deprivation, two bioenergetic challenges of fundamental importance in the evolution of the brains of all mammals, including humans...
November 28, 2016: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/27906632/changes-in-cardiac-nucleotide-metabolism-in-huntington-s-disease
#6
Marta Toczek, Barbara Kutryb-Zajac, Paulina Zukowska, Ewa M Slominska, Mark Isalan, Michal Mielcarek, Ryszard T Smolenski
Huntington's disease (HD) is a monogenic neurodegenerative disorder with a significant peripheral component to the disease pathology. This includes an HD-related cardiomyopathy, with an unknown pathological mechanism. In this study, we aimed to define changes in the metabolism of cardiac nucleotides using the well-established R6/2 mouse model. In particular, we focused on measuring the activity of enzymes that control ATP and other adenine nucleotides in the cardiac pool, including eNTPD, AMPD, e5'NT, ADA, and PNP...
December 2016: Nucleosides, Nucleotides & Nucleic Acids
https://www.readbyqxmd.com/read/27906525/the-clinical-utility-of-qsm-disease-diagnosis-medical-management-and-surgical-planning
#7
REVIEW
Sarah Eskreis-Winkler, Yan Zhang, Jingwei Zhang, Zhe Liu, Alexey Dimov, Ajay Gupta, Yi Wang
Quantitative susceptibility mapping (QSM) is an MR technique that depicts and quantifies magnetic susceptibility sources. Mapping iron, the dominant susceptibility source in the brain, has many important clinical applications. Herein, we review QSM applications in the diagnosis, medical management, and surgical treatment of disease. To assist in early disease diagnosis, QSM can identify elevated iron levels in the motor cortex of amyotrophic lateral sclerosis patients, in the substantia nigra of Parkinson's disease (PD) patients, in the globus pallidus, putamen, and caudate of Huntington's disease patients, and in the basal ganglia of Wilson's disease patients...
December 1, 2016: NMR in Biomedicine
https://www.readbyqxmd.com/read/27903442/are-micrornas-true-sensors-of-ageing-and-cellular-senescence
#8
REVIEW
Justin Williams, Flint Smith, Subodh Kumar, Murali Vijayan, P Hemachandra Reddy
All living beings are programmed to death due to aging and age-related processes. Aging is a normal process of every living species. While all cells are inevitably progressing towards death, many disease processes accelerate the aging process, leading to senescence. Pathologies such as Alzheimer's disease, Parkinson's disease, multiple sclerosis, amyotrophic lateral sclerosis, Huntington's disease, cardiovascular disease, cancer, and skin diseases have been associated with deregulated aging. Healthy aging can delay onset of all age-related diseases...
November 26, 2016: Ageing Research Reviews
https://www.readbyqxmd.com/read/27902448/rasgrp1-promotes-amphetamine-induced-motor-behavior-through-a-rhes-interaction-network-rhesactome-in-the-striatum
#9
Neelam Shahani, Supriya Swarnkar, Vincenzo Giovinazzo, Jenny Morgenweck, Laura M Bohn, Catherina Scharager-Tapia, Bruce Pascal, Pablo Martinez-Acedo, Kshitij Khare, Srinivasa Subramaniam
The striatum of the brain coordinates motor function. Dopamine-related drugs may be therapeutic to patients with striatal neurodegeneration, such as Huntington's disease (HD) and Parkinson's disease (PD), but these drugs have unwanted side effects. In addition to stimulating the release of norepinephrine, amphetamines, which are used for narcolepsy and attention-deficit/hyperactivity disorder (ADHD), trigger dopamine release in the striatum. The guanosine triphosphatase Ras homolog enriched in the striatum (Rhes) inhibits dopaminergic signaling in the striatum, is implicated in HD and L-dopa-induced dyskinesia, and has a role in striatal motor control...
November 15, 2016: Science Signaling
https://www.readbyqxmd.com/read/27899419/progressive-cl-channel-defects-reveal-disrupted-skeletal-muscle-maturation-in-r6-2-huntington-s-mice
#10
Daniel R Miranda, Monica Wong, Shannon H Romer, Cynthia McKee, Gabriela Garza-Vasquez, Alyssa C Medina, Volker Bahn, Andrew D Steele, Robert J Talmadge, Andrew A Voss
Huntington's disease (HD) patients suffer from progressive and debilitating motor dysfunction. Previously, we discovered reduced skeletal muscle chloride channel (ClC-1) currents, inwardly rectifying potassium (Kir) channel currents, and membrane capacitance in R6/2 transgenic HD mice. The ClC-1 loss-of-function correlated with increased aberrant mRNA processing and decreased levels of full-length ClC-1 mRNA (Clcn1 gene). Physiologically, the resulting muscle hyperexcitability may help explain involuntary contractions of HD...
November 29, 2016: Journal of General Physiology
https://www.readbyqxmd.com/read/27895927/expansion-mosaicism-and-interruption-mechanisms-of-the-cag-repeat-mutation-in-spinocerebellar-ataxia-type-1
#11
REVIEW
Cara Kraus-Perrotta, Sarita Lagalwar
Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant neurodegenerative disorder that primarily affects the cerebellum and brainstem. The genetic mutation is an expansion of CAG trinucleotide repeats within the coding region of the ataxin-1 gene, characterizing SCA1 as a polyglutamine expansion disease like Huntington's. As with most polyglutamine expansion diseases, SCA1 follows the rules of genetic anticipation: the larger the expansion, the earlier and more rapid the symptoms. Unlike the majority of polyglutamine expansion diseases, the presence of histidine interruptions within the polyglutamine tract of ataxin-1 protein can prevent or mitigate disease...
2016: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/27895381/reactive-oxygen-species-induce-neurite-degeneration-before-induction-of-cell-death
#12
Koji Fukui
Reactive oxygen species (ROS) induce neuronal cell death in a time- and concentration-dependent manner. Treatment of cultured cells with a low concentration of hydrogen peroxide induces neurite degeneration, but not cell death. Neurites (axons and dendrites) are vulnerable to ROS. Neurite degeneration (shrinkage, accumulation, and fragmentation) has been found in neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease, and Huntington's disease. However, the mechanism of ROS-related neurite degeneration is not fully understood...
November 2016: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/27892808/self-reported-impulsivity-in-huntington-s-disease-patients-and-relationship-to-executive-dysfunction-and-reward-responsiveness
#13
Patricia L Johnson, Geoffrey F Potts, Juan Sanchez-Ramos, Cynthia R Cimino
INTRODUCTION: Few studies have directly investigated impulsivity in Huntington's disease (HD) despite known changes in dopaminergic and frontal functioning, changes that have been associated with impulsivity in other disorders and in the normal population. This study sought to further categorize impulsivity in HD through examining differences in self-reported impulsivity between community controls and HD patients, the relationship between executive dysfunction and impulsivity, and the relationship of a reward/punishment behavioral inhibition task in relation to these self-report measures...
November 28, 2016: Journal of Clinical and Experimental Neuropsychology
https://www.readbyqxmd.com/read/27892614/validation-of-a-prognostic-index-for-huntington-s-disease
#14
Jeffrey D Long, Douglas R Langbehn, Sarah J Tabrizi, Bernhard G Landwehrmeyer, Jane S Paulsen, John Warner, Cristina Sampaio
BACKGROUND: Characterizing progression in Huntington's disease is important for study the natural course and selecting appropriate participants for clinical trials. OBJECTIVES: The aim was to develop a prognostic index for motor diagnosis in Huntington's disease and examine its predictive performance in external observational studies. METHODS: The prediagnosis Neuro-biological Predictors of Huntington's Disease study (N = 945 gene-positive) was used to select a Cox regression model for computing a prognostic index...
November 28, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27892468/somatic-increase-of-cct8-mimics-proteostasis-of-human-pluripotent-stem-cells-and-extends-c-elegans-lifespan
#15
Alireza Noormohammadi, Amirabbas Khodakarami, Ricardo Gutierrez-Garcia, Hyun Ju Lee, Seda Koyuncu, Tim König, Christina Schindler, Isabel Saez, Azra Fatima, Christoph Dieterich, David Vilchez
Human embryonic stem cells can replicate indefinitely while maintaining their undifferentiated state and, therefore, are immortal in culture. This capacity may demand avoidance of any imbalance in protein homeostasis (proteostasis) that would otherwise compromise stem cell identity. Here we show that human pluripotent stem cells exhibit enhanced assembly of the TRiC/CCT complex, a chaperonin that facilitates the folding of 10% of the proteome. We find that ectopic expression of a single subunit (CCT8) is sufficient to increase TRiC/CCT assembly...
November 28, 2016: Nature Communications
https://www.readbyqxmd.com/read/27891320/the-association-of-vdac-with-cell-viability-of-pc12-model-of-huntington-s-disease
#16
Andonis Karachitos, Daria Grobys, Klaudia Kulczyńska, Adrian Sobusiak, Hanna Kmita
It is becoming increasingly apparent that mitochondria dysfunction plays an important role in the pathogenesis of Huntington's disease (HD), but the underlying mechanism is still elusive. Thus, there is a still need for further studies concerning the upstream events in the mitochondria dysfunction that could contribute to cell death observed in HD. Taking into account the fundamental role of the voltage-dependent anion-selective channel (VDAC) in mitochondria functioning, it is reasonable to consider the channel as a crucial element in HD etiology...
2016: Frontiers in Oncology
https://www.readbyqxmd.com/read/27890852/adenocarcinoma-arising-from-an-end-ileostomy-in-a-patient-with-familial-adenomatous-polyposis-fap
#17
Justin T Huntington, Peter P Stanich, Alan E Harzman
No abstract text is available yet for this article.
November 24, 2016: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/27890638/aberrant-base-excision-repair-pathway-of-oxidatively-damaged-dna-implications-for-degenerative-diseases
#18
Ibtissam Talhaoui, Bakhyt T Matkarimov, Thierry Tchenio, Dmitry O Zharkov, Murat K Saparbaev
In cellular organisms composition of DNA is constrained to only four nucleobases A, G, T and C, except for minor DNA base modifications such as methylation which serves for defence against foreign DNA or gene expression regulation. Interestingly, this severe evolutionary constraint among other things demands DNA repair systems to discriminate between regular and modified bases. DNA glycosylases specifically recognize and excise damaged bases among vast majority of regular bases in the base excision repair (BER) pathway...
November 24, 2016: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/27890093/3-nitropropionic-acid-production-by-the-endophytic-diaporthe-citri-molecular-taxonomy-chemical-characterization-and-quantification-under-ph-variation
#19
Julio Cesar Polonio, Marcos Alessandro Dos Santos Ribeiro, Sandro Augusto Rhoden, Maria Helena Sarragiotto, João Lúcio Azevedo, João Alencar Pamphile
3-nitropropionic acid (3-NPA) is a nitrogenated compound produced by plants and fungi and has been associated with poisoning episodes in humans, animals, and to induction of Huntington disease symptoms in rats. The production of 3-NPA by endophytes has been reported, but the function and biosynthesis are not well-defined. The specie of endophytic strain G-01 was confirmed as Diaporthe citri using a multilocus sequence analysis, and was verified different concentrations of 3-NPA produced at different initial pHs by these strain...
December 2016: Fungal Biology
https://www.readbyqxmd.com/read/27889578/molecular-neurobiology-of-mtor
#20
REVIEW
Katarzyna Switon, Katarzyna Kotulska, Aleksandra Janusz-Kaminska, Justyna Zmorzynska, Jacek Jaworski
Mammalian/mechanistic target of rapamycin (mTOR) is a serine-threonine kinase that controls several important aspects of mammalian cell function. mTOR activity is modulated by various intra- and extracellular factors; in turn, mTOR changes rates of translation, transcription, protein degradation, cell signaling, metabolism, and cytoskeleton dynamics. mTOR has been repeatedly shown to participate in neuronal development and the proper functioning of mature neurons. Changes in mTOR activity are often observed in nervous system diseases, including genetic diseases (e...
November 23, 2016: Neuroscience
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