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Sylvie Bannwarth et al.
Brain : a journal of neurology, 137(Pt 8), 2329-2345 (2014-06-18)
Mitochondrial DNA instability disorders are responsible for a large clinical spectrum, among which amyotrophic lateral sclerosis-like symptoms and frontotemporal dementia are extremely rare. We report a large family with a late-onset phenotype including motor neuron disease, cognitive decline resembling frontotemporal
Lu Gao et al.
The Journal of clinical investigation, 125(7), 2808-2824 (2015-06-23)
The precise mechanisms that lead to parturition are incompletely defined. Surfactant protein-A (SP-A), which is secreted by fetal lungs into amniotic fluid (AF) near term, likely provides a signal for parturition; however, SP-A-deficient mice have only a relatively modest delay
Sébastien M Labbé et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 29(5), 2046-2058 (2015-02-15)
The present study was designed to investigate the effects of cold on brown adipose tissue (BAT) energy substrate utilization in vivo using the positron emission tomography tracers [(18)F]fluorodeoxyglucose (glucose uptake), 14(R,S)-[(18)F]fluoro-6-thia-heptadecanoic acid [nonesterified fatty acid (NEFA) uptake], and [(11)C]acetate (oxidative
Eros Quarta et al.
The Journal of comparative neurology, 523(11), 1622-1638 (2015-02-17)
Amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease primarily characterized by motor neuron death, causes damages beyond motor-related areas. In particular, cognitive impairments and hippocampal damage have been reported in ALS patients. We investigated spatial navigation learning and hippocampal interneurons
David L Wang et al.
PloS one, 10(3), e0121826-e0121826 (2015-03-27)
A new type of technology in proteomics was developed in order to separate a complex protein mixture and analyze protein functions systematically. The technology combines the ability of two-dimensional gel electrophoresis (2-DE) to separate proteins with a protein elution plate
Lester Carter et al.
Biophysical journal, 109(4), 793-805 (2015-08-20)
Aberrant self-assembly, induced by structural misfolding of the prion proteins, leads to a number of neurodegenerative disorders. In particular, misfolding of the mostly α-helical cellular prion protein (PrP(C)) into a β-sheet-rich disease-causing isoform (PrP(Sc)) is the key molecular event in
Valentina Fodale et al.
PloS one, 9(12), e112262-e112262 (2014-12-03)
In Huntington's disease, expansion of a CAG triplet repeat occurs in exon 1 of the huntingtin gene (HTT), resulting in a protein bearing>35 polyglutamine residues whose N-terminal fragments display a high propensity to misfold and aggregate. Recent data demonstrate that
Helen Künstlinger et al.
Oncotarget, 6(24), 20215-20230 (2015-06-04)
Myxoid liposarcomas account for more than one third of liposarcomas and about 10% of all adult soft tissue sarcomas. The tumors are characterized by specific chromosomal translocations leading to the chimeric oncogenes FUS-DDIT3 or EWS1R-DDIT3. The encoded fusion proteins act
Kentu Lassiter et al.
American journal of physiology. Regulatory, integrative and comparative physiology, 308(3), R173-R187 (2014-12-17)
Orexin A and B, orexigenic peptides produced primarily by the lateral hypothalamus that signal through two G protein-coupled receptors, orexin receptors 1/2, have been implicated in the regulation of several physiological processes in mammals. In avian (nonmammalian vertebrates) species; however
Elizabeth A Reznikov et al.
The Journal of nutrition, 144(9), 1401-1408 (2014-07-25)
Lactoferrin is a bioactive milk protein that stimulates cell proliferation in vitro; however, limited in vivo evidence exists to allow lactoferrin to be incorporated into infant formula. Herein, the effect of dietary bovine lactoferrin (bLF) on neonatal intestinal growth and
Midori Suenaga et al.
Biochimica et biophysica acta, 1854(8), 1010-1018 (2015-05-10)
Impaired clearance of soluble Aβ (amyloid-β) promotes Aβ aggregation in brains with Alzheimer's disease (AD), while apolipoprotein-E (ApoE) in microglia mediates Aβ clearance. We studied the protease responsible for ApoE(4) degradation in human peripheral monocyte extracts, which are from the
Violeta Gomez-Rodriguez et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 29(3), 960-972 (2014-11-22)
We studied the role of matrix metalloproteinase-10 (MMP-10) during skeletal muscle repair after ischemia using a model of femoral artery excision in wild-type (WT) and MMP-10 deficient (Mmp10(-/-)) mice. Functional changes were analyzed by small animal positron emission tomography and
Andreas Herchenhan et al.
The Journal of biological chemistry, 290(26), 16440-16450 (2015-05-17)
Lysyl oxidases (LOXs) are a family of copper-dependent oxido-deaminases that can modify the side chain of lysyl residues in collagen and elastin, thereby leading to the spontaneous formation of non-reducible aldehyde-derived interpolypeptide chain cross-links. The consequences of LOX inhibition in
Thao T Nguyen et al.
FEBS letters, 589(17), 2186-2193 (2015-07-21)
In plants and fungi the plasma membrane proton pump generates a large proton-motive force that performs essential functions in many processes, including solute transport and the control of cell elongation. Previous studies in yeast and higher plants have indicated that
Afrouz Yousefi et al.
International journal of pharmaceutics, 472(1-2), 175-184 (2014-06-21)
Angiogenesis is one of the hallmarks of cancer which renders it an attractive target for therapy of malignancies. Tumor growth suppression can be achieved by inhibiting angiogenesis since it would deprive tumor cells of oxygen and vital nutrients. Activation of
Zhe Wang et al.
ACS nano, 9(7), 6683-6695 (2015-05-29)
Recent advances in neural stem cell (NSC) transplantation have led to an inspiring progress in alleviating central nervous system (CNS) damages and restoring brain functions from diseases or injuries. One challenge of NSC transplantation is directed differentiation of transplanted NSCs
Jacob C Schwartz et al.
Molecular biology of the cell, 25(17), 2571-2578 (2014-07-11)
Mutations in the RNA-binding protein FUS have been shown to cause the neurodegenerative disease amyotrophic lateral sclerosis (ALS). We investigate whether mutant FUS protein in ALS patient-derived fibroblasts affects normal FUS functions in the nucleus. We investigated fibroblasts from two
Åsa M E Winther et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 35(44), 14756-14770 (2015-11-06)
Many endocytic proteins accumulate in the reserve pool of synaptic vesicles (SVs) in synapses and relocalize to the endocytic periactive zone during neurotransmitter release. Currently little is known about their functions outside the periactive zone. Here we show that in
Diana L Castillo-Carranza et al.
Journal of Alzheimer's disease : JAD, 40 Suppl 1, S97-S111 (2014-03-08)
Neurodegenerative disease is one of the greatest health crises in the world and as life expectancy rises, the number of people affected will continue to increase. The most common neurodegenerative disease, Alzheimer's disease, is a tauopathy, characterized by the presence
Laetitia Francelle et al.
Human molecular genetics, 24(6), 1563-1573 (2014-11-16)
The mechanisms underlying preferential atrophy of the striatum in Huntington's disease (HD) are unknown. One hypothesis is that a set of gene products preferentially expressed in the striatum could determine the particular vulnerability of this brain region to mutant huntingtin
Judith Müller et al.
Nature communications, 5, 5712-5712 (2014-12-17)
Increased expression of the Microphthalmia-associated transcription factor (MITF) contributes to melanoma progression and resistance to BRAF pathway inhibition. Here we show that the lack of MITF is associated with more severe resistance to a range of inhibitors, while its presence
Aneesh Vijayan et al.
PloS one, 10(7), e0133595-e0133595 (2015-07-25)
In the HIV vaccine field, there is a need to produce highly immunogenic forms of the Env protein with the capacity to trigger broad B and T-cell responses. Here, we report the generation and characterization of a chimeric HIV-1 gp120
Mette D Mosgaard et al.
European journal of pharmaceutics and biopharmaceutics : official journal of Arbeitsgemeinschaft fur Pharmazeutische Verfahrenstechnik e.V, 94, 493-500 (2015-07-15)
To develop a high-throughput in vitro intestinal lipolysis (HTP) model, without any means of pH-stat-titration, to enable a fast evaluation of lipid-based drug delivery systems (LbDDS). The HTP model was compared to the traditionally used dynamic in vitro lipolysis (DIVL)
Sean M Hartig et al.
Molecular endocrinology (Baltimore, Md.), 29(9), 1320-1333 (2015-07-21)
Insulin resistance and type 2 diabetes mellitus (T2DM) result from an inability to efficiently store and catabolize surplus energy in adipose tissue. Subcutaneous adipocytes protect against insulin resistance and T2DM by coupling differentiation with the induction of brown fat gene
Cailing Liu et al.
Investigative ophthalmology & visual science, 55(9), 5551-5560 (2014-08-02)
To investigate the role of DJ-1 in Nrf2-regulated antioxidant defense in corneal endothelial cells (CECs) at baseline and in response to ultraviolet A (UV-A)-induced oxidative stress. DJ-1-deficient CECs were obtained by transfection of an immortalized normal human corneal endothelial cell
H Zhong et al.
Gene therapy, 22(8), 619-627 (2015-05-13)
Loss of SPATA7 function causes the pathogenesis of Leber congenital amaurosis and retinitis pigmentosa. Spata7 knockout mice mimic human SPATA7-related retinal disease with apparent photoreceptor degeneration observed as early as postnatal day 15 (P15). To test the efficacy of adeno-associated
Yunhong Huang et al.
Science translational medicine, 7(309), 309ra164-309ra164 (2015-10-16)
The orphan G protein (heterotrimeric guanine nucleotide-binding protein)-coupled receptor (GPCR) GPR3 regulates activity of the γ-secretase complex in the absence of an effect on Notch proteolysis, providing a potential therapeutic target for Alzheimer's disease (AD). However, given the vast resources
Mijeong Kim et al.
International journal of genomics, 2015, 238704-238704 (2015-12-01)
In rice, the stage of development most sensitive to high temperature stress is flowering, and exposure at this stage can result in spikelet sterility, thereby leading to significant yield losses. In this study, protein expression patterns of rice anthers from
Bing Han et al.
World journal of gastroenterology, 21(33), 9727-9735 (2015-09-12)
To confirm the anti-invasion and anti-migration effects of down-regulation of Notch1 combined with interleukin (IL)-24 in hepatocellular carcinoma (HCC) cells. γ-secretase inhibitors (GSIs) were used to down-regulate Notch1. HepG2 and SMMC7721 cells were seeded in 96-well plates and treated with
Adrian T Billeter et al.
Surgery, 158(3), 646-654 (2015-06-10)
Hypothermia is a well-known risk factor for postoperative complications because it prolongs the monocyte inflammatory response. The purpose of this study was to investigate whether temperature-activated ion channels (transient receptor protein channels [TRP] A1 and V1) mediate the effects of
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