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https://www.readbyqxmd.com/read/26086378/repeat-associated-non-aug-translation-ran-translation-dependent-on-sequence-downstream-of-the-atxn2-cag-repeat
#1
Daniel R Scoles, Mi H T Ho, Warunee Dansithong, Lance T Pflieger, Lance W Petersen, Khanh K Thai, Stefan M Pulst
Spinocerebellar ataxia type 2 (SCA2) is a progressive autosomal dominant disorder caused by the expansion of a CAG tract in the ATXN2 gene. The SCA2 disease phenotype is characterized by cerebellar atrophy, gait ataxia, and slow saccades. ATXN2 mutation causes gains of toxic and normal functions of the ATXN2 gene product, ataxin-2, and abnormally slow Purkinje cell firing frequency. Previously we investigated features of ATXN2 controlling expression and noted expression differences for ATXN2 constructs with varying CAG lengths, suggestive of repeat associated non-AUG translation (RAN translation)...
2015: PloS One
https://www.readbyqxmd.com/read/24521198/single-amino-acid-repeats-connect-viruses-to-neurodegeneration
#2
Guglielmo Lucchese, Darja Kanduc
We report on a high level of octapeptide matching between HCV, HIV-2, MPV, MUV, EBV, HHV-6, and CMV, and human brain antigens that, when altered, have been specifically associated with neuropathologies such as amyotrophic lateral sclerosis, spinocerebellar ataxia, frontotemporal degeneration, Huntington disease, Parkinson disease, cognitive impairment, aphasia and oculomotor apraxia. Quantitatively, the extent of the viral octapeptide sharing with neurodegeneration- associated proteins is in excess when analyzed in a stochastic expectation context...
2014: Current Drug Discovery Technologies
https://www.readbyqxmd.com/read/24361760/generation-of-a-neurodegenerative-disease-mouse-model-using-lentiviral-vectors-carrying-an-enhanced-synapsin-i-promoter
#3
Yasunori Matsuzaki, Miho Oue, Hirokazu Hirai
BACKGROUND: Certain inherited progressive neurodegenerative disorders, such as spinocerebellar ataxia (SCA), affect neurons in large areas of the central nervous system (CNS). The selective expression of disease-causing and therapeutic genes in susceptible regions and cell types is critical for the generation of animal models and development of gene therapies for these diseases. Previous studies have demonstrated the advantages of the short synapsin I (SynI) promoter (0.5 kb) as a neuron-specific promoter for robust transgene expression...
February 15, 2014: Journal of Neuroscience Methods
https://www.readbyqxmd.com/read/23656738/markers-of-nonselective-and-specific-nk-cell-activation
#4
Leslie A Fogel, Michel M Sun, Theresa L Geurs, Leonidas N Carayannopoulos, Anthony R French
NK cell activation is controlled by the integration of signals from cytokine receptors and germline-encoded activation and inhibitory receptors. NK cells undergo two distinct phases of activation during murine CMV (MCMV) infection: a nonselective phase mediated by proinflammatory cytokines and a specific phase driven by signaling through Ly49H, an NK cell activation receptor that recognizes infected cells. We sought to delineate cell surface markers that could distinguish NK cells that had been activated nonselectively from those that had been specifically activated through NK cell receptors...
June 15, 2013: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/20732421/human-cytomegalovirus-ul97-kinase-prevents-the-deposition-of-mutant-protein-aggregates-in-cellular-models-of-huntington-s-disease-and-ataxia
#5
Cristy Tower, Lianwu Fu, Rachel Gill, Mark Prichard, Mathieu Lesort, Elizabeth Sztul
The presence of aggregates of abnormally expanded polyglutamine (polyQ)-containing proteins are a pathological hallmark of a number of neurodegenerative diseases including Huntington's disease (HD) and spinocerebellar ataxia-3 (SCA3). Previous studies in cellular, Drosophila, and mouse models of HD and SCA have shown that neurodegeneration can be prevented by manipulations that inhibit polyQ aggregation. We have shown that the UL97 kinase of the human cytomegalovirus (HCMV) prevents aggregation of the pp71 and pp65 viral tegument proteins...
January 2011: Neurobiology of Disease
https://www.readbyqxmd.com/read/17339451/flt3-ligand-expands-lymphoid-progenitors-prior-to-recovery-of-thymopoiesis-and-accelerates-t-cell-reconstitution-after-bone-marrow-transplantation
#6
Evert-Jan Wils, Eric Braakman, Georges M G M Verjans, Elwin J C Rombouts, Annoek E C Broers, Hubert G M Niesters, Gerard Wagemaker, Frank J T Staal, Bob Löwenberg, Hergen Spits, Jan J Cornelissen
Deficient thymopoiesis and retarded recovery of newly developed CD4(+) T cells is one of the most important determinants of impaired immunocompetence after hemopoietic stem cell transplantation. Here we evaluated whether Fms-like tyrosine kinase 3 (Flt3) ligand (FL) alone or combined with IL-7 affects T cell recovery, thymopoiesis, and lymphoid progenitor expansion following bone marrow transplantation in immunodeficient mice. FL strongly accelerated and enhanced the recovery of peripheral T cells after transplantation of a low number of bone marrow cells...
March 15, 2007: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/16046529/cytomegalovirus-mck-2-controls-mobilization-and-recruitment-of-myeloid-progenitor-cells-to-facilitate-dissemination
#7
Satoshi Noda, Shirley A Aguirre, Andrew Bitmansour, Janice M Brown, Timothy E Sparer, Jing Huang, Edward S Mocarski
Murine cytomegalovirus encodes a secreted, pro-inflammatory chemokine-like protein, MCK-2, that recruits leukocytes and facilitates viral dissemination. We have shown that MCK-2-enhanced recruitment of myelomonocytic leukocytes with an immature phenotype occurs early during infection and is associated with efficient viral dissemination. Expression of MCK-2 drives the mobilization of a population of leukocytes from bone marrow that express myeloid marker Mac-1 (CD11b), intermediate levels of Gr-1 (Ly6 G/C), platelet-endothelial-cell adhesion molecule-1 (PECAM-1, CD31), together with heterogeneous levels of stem-cell antigen-1 (Sca-1, Ly-6 A /E)...
January 1, 2006: Blood
https://www.readbyqxmd.com/read/11823032/lentiviral-vector-mediated-transduction-of-murine-cd34-hematopoietic-stem-cells
#8
Satoko Tahara-Hanaoka, Kazuhiro Sudo, Hideo Ema, Hiroyuki Miyoshi, Hiromitsu Nakauchi
OBJECTIVE: Efficient gene transfer into murine hematopoietic stem cells (HSCs) provides a powerful tool for exploring hematopoietic stem cell biology. In this study, we evaluated the efficiency of lentiviral vector-mediated gene transfer into murine CD34(-/low)c-Kit(+)Sca-1(+)Lin(-) (CD34(-) KSL) cells that are highly enriched for HSCs. MATERIALS AND METHODS: FACS-sorted CD34(-) KSL cells were transduced with the vesicular stomatitis virus G glycoprotein-pseudotyped HIV-1-based lentiviral vector containing the green fluorescent protein (GFP) gene under the control of the cytomegalovirus promoter, and then 50 transduced cells were transplanted into lethally irradiated mice...
January 2002: Experimental Hematology
https://www.readbyqxmd.com/read/9918835/identification-of-the-physiological-promoter-for-spinocerebellar-ataxia-2-gene-reveals-a-cpg-island-for-promoter-activity-situated-into-the-exon-1-of-this-gene-and-provides-data-about-the-origin-of-the-nonmethylated-state-of-these-types-of-islands
#9
COMPARATIVE STUDY
J Aguiar, S Santurlidis, J Nowok, C Alexander, D Rudnicki, S Gispert, W Schulz, G Auburger
In order to further use the spinocerebellar ataxia 2 (SCA2) promoter for transgenic mice models of "CAG repeat" neurodegeneration, different fragments of this 5' end were ligated into pGL3-Luc plasmid to obtain the better promoter-activity of the physiological promoter for SCA2. Base-par composition of the SCA2-5' region, and promoter prediction algorithms such as TSSW and TSSG, together with the high firefly luciferase expression after 48 hours of transient transfection in mammalian cells lines, showed a typical CpG island for promoter-activity...
January 19, 1999: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/9160661/fas-mediated-apoptosis-of-the-hematopoietic-progenitor-cells-in-mice-infected-with-murine-cytomegalovirus
#10
T Mori, K Ando, K Tanaka, Y Ikeda, Y Koga
The effects of cytomegalovirus (CMV) infection on hematopoietic progenitor cells in vivo were investigated to elucidate the pathogenesis of CMV-induced myelosuppression. BALB/c mice were inoculated with 0.2LD50 of murine CMV (MCMV). Lineage marker negative, c-kit positive (Lin-c-kit+) and Lin-CD34+ cells, which are both phenotypically defined as hematopoietic progenitor cells, showed a significant reduction in number on day 3 postinfection (pi). Moreover, the reduction in the number of day-14 colony-forming units-spleen (CFU-S), another indicator to identify hematopoietic progenitor cells, was noted on day 3 pi...
May 15, 1997: Blood
https://www.readbyqxmd.com/read/7688711/characterization-of-thymic-involution-induced-by-murine-cytomegalovirus-infection
#11
COMPARATIVE STUDY
P Price, S D Olver, A E Gibbons, H K Teo, G R Shellam
Infection of BALB/c mice with murine cytomegalovirus (MCMV) decreased the numbers of cells recovered from the thymus by 80-90% after 4-7 days, although less than 10 thymocytes per million were productively infected with the virus. A loss of cortical thymocytes was evident in histologic sections and correlated with depletion of CD4+ CD8+ cells. Thymic involution was minimal in C57BL/6 mice. This resistance was not H-2b-associated, as BALB.B (H-2b) mice were severely affected. In CXB recombinant inbred mice, thymic involution and MCMV replication co-segregated with atrophy and infection of the spleen and bone marrow...
June 1993: Immunology and Cell Biology
https://www.readbyqxmd.com/read/7541663/analysis-of-hematopoietic-stem-and-progenitor-cell-populations-in-cytomegalovirus-infected-mice
#12
COMPARATIVE STUDY
A E Gibbons, P Price, G R Shellam
We have studied the effects of murine cytomegalovirus (MCMV) infection on bone marrow stem and progenitor cell populations to find an explanation for the defects in hematopoiesis that accompany CMV infections in patients. Sublethal MCMV infection of BALB/c mice resulted in a 5- to 10-fold decrease in the numbers of myeloid (colony-forming unit-granulocyte-macrophage [CFU-GM]) and erythroid (burst-forming unit-erythroid [BFU-E]) progenitor cells in the marrow, but not in primitive myeloerythroid progenitor cell (colony-forming unit-spleen [CFU-S]) numbers...
July 15, 1995: Blood
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