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  • 1
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    Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) [Medical Sciences] (2012)
    National Academy of Sciences
    Details
    Publication Date: 2012-02-01
    Description: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a childhood-onset neurological disease resulting from mutations in the SACS gene encoding sacsin, a 4,579-aa protein of unknown function. Originally identified as a founder disease in Québec, ARSACS is now recognized worldwide. Prominent features include pyramidal spasticity and cerebellar ataxia, but the underlying pathology and pathophysiological mechanisms are unknown. We have generated an animal model for ARSACS, sacsin knockout mice, that display age-dependent neurodegeneration of cerebellar Purkinje cells. To explore the pathophysiological basis for this observation, we examined the cell biological properties of sacsin. We show that sacsin localizes to mitochondria in non-neuronal cells and primary neurons and that it interacts with dynamin-related protein 1, which participates in mitochondrial fission. Fibroblasts from ARSACS patients show a hyperfused mitochondrial network, consistent with defects in mitochondrial fission. Sacsin knockdown leads to an overly interconnected and functionally impaired mitochondrial network, and mitochondria accumulate in the soma and proximal dendrites of sacsin knockdown neurons. Disruption of mitochondrial transport into dendrites has been shown to lead to abnormal dendritic morphology, and we observe striking alterations in the organization of dendritic fields in the cerebellum of knockout mice that precedes Purkinje cell death. Our data identifies mitochondrial dysfunction/mislocalization as the likely cellular basis for ARSACS and indicates a role for sacsin in regulation of mitochondrial dynamics.
    Print ISSN: 0027-8424
    Electronic ISSN: 1091-6490
    Topics: Biology , Medicine , Natural Sciences in General
    Published by National Academy of Sciences
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  • 2
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    Involvement of mammalian Mus81 in genome integrity and tumor suppression (2004)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 2004-06-19
    Description: Mus81-Eme1 endonuclease has been implicated in the rescue of stalled replication forks and the resolution of meiotic recombination intermediates in yeast. We used gene targeting to study the physiological requirements of Mus81 in mammals. Mus81-/- mice are viable and fertile, which indicates that mammalian Mus81 is not essential for recombination processes associated with meiosis. Mus81-deficient mice and cells were hypersensitive to the DNA cross-linking agent mitomycin C but not to gamma-irradiation. Remarkably, both homozygous Mus81-/- and heterozygous Mus81+/- mice exhibited a similar susceptibility to spontaneous chromosomal damage and a profound and equivalent predisposition to lymphomas and other cancers. These studies demonstrate a critical role for the proper biallelic expression of the mammalian Mus81 in the maintenance of genomic integrity and tumor suppression.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉McPherson, John Peter -- Lemmers, Benedicte -- Chahwan, Richard -- Pamidi, Ashwin -- Migon, Eva -- Matysiak-Zablocki, Elzbieta -- Moynahan, Mary Ellen -- Essers, Jeroen -- Hanada, Katsuhiro -- Poonepalli, Anuradha -- Sanchez-Sweatman, Otto -- Khokha, Rama -- Kanaar, Roland -- Jasin, Maria -- Hande, M Prakash -- Hakem, Razqallah -- New York, N.Y. -- Science. 2004 Jun 18;304(5678):1822-6.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Ontario Cancer Institute, 620 University Avenue, Suite 706, Toronto, Ontario, Canada M5G 2C1.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/15205536" target="_blank"〉PubMed〈/a〉
    Keywords: Alleles ; Animals ; Chromosome Aberrations ; DNA Damage ; DNA-Binding Proteins/*genetics/*physiology ; Embryo, Mammalian/cytology ; Embryonic and Fetal Development ; *Endonucleases ; Gamma Rays ; Gene Targeting ; Genetic Predisposition to Disease ; *Genome ; *Genomic Instability ; Heterozygote ; Lymphoma/etiology/genetics/pathology ; Meiosis ; Mice ; Mitomycin/pharmacology ; Neoplasms/etiology/*genetics ; Recombination, Genetic ; Saccharomyces cerevisiae Proteins ; Sister Chromatid Exchange ; Stem Cells ; T-Lymphocytes/physiology
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
    Published by American Association for the Advancement of Science (AAAS)
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  • 3
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    Molecular cloning, chromosomal mapping, and functional expression of human brain glutamate receptors. (1992)
    National Academy of Sciences
    Details
    Publication Date: 1992-02-15
    Print ISSN: 0027-8424
    Electronic ISSN: 1091-6490
    Topics: Biology , Medicine , Natural Sciences in General
    Published by National Academy of Sciences
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  • 4
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    Fanconi anemia signaling and Mus81 cooperate to safeguard development and crosslink repair (2014)
    Oxford University Press
    Details
    Publication Date: 2014-09-02
    Description: Individuals with Fanconi anemia (FA) are susceptible to bone marrow failure, congenital abnormalities, cancer predisposition and exhibit defective DNA crosslink repair. The relationship of this repair defect to disease traits remains unclear, given that crosslink sensitivity is recapitulated in FA mouse models without most of the other disease-related features. Mice deficient in Mus81 are also defective in crosslink repair, yet MUS81 mutations have not been linked to FA. Using mice deficient in both Mus81 and the FA pathway protein FancC, we show both proteins cooperate in parallel pathways, as concomitant loss of FancC and Mus81 triggered cell-type-specific proliferation arrest, apoptosis and DNA damage accumulation in utero . Mice deficient in both FancC and Mus81 that survived to birth exhibited growth defects and an increased incidence of congenital abnormalities. This cooperativity of FancC and Mus81 in developmental outcome was also mirrored in response to crosslink damage and chromosomal integrity. Thus, our findings reveal that both pathways safeguard against DNA damage from exceeding a critical threshold that triggers proliferation arrest and apoptosis, leading to compromised in utero development.
    Print ISSN: 0305-1048
    Electronic ISSN: 1362-4962
    Topics: Biology
    Published by Oxford University Press
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  • 5
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    2,3,7,8-Tetrachlorodibenzo-p-dioxin poly(ADP-ribose) polymerase (TiPARP, ARTD14) is a mono-ADP-ribosyltransferase and repressor of aryl hydrocarbon receptor transactivation (2013)
    Oxford University Press
    Details
    Publication Date: 2013-02-02
    Description: 2,3,7,8-Tetrachlorodibenzo- p -dioxin (TCDD)-inducible poly(ADP-ribose) polymerase (TiPARP/ARTD14) is a member of the PARP family and is regulated by the aryl hydrocarbon receptor (AHR); however, little is known about TiPARP function. In this study, we examined the catalytic function of TiPARP and determined its role in AHR transactivation. We observed that TiPARP exhibited auto-mono-ADP-ribosyltransferase activity and ribosylated core histones. RNAi-mediated knockdown of TiPARP in T-47D breast cancer and HuH-7 hepatoma cells increased TCDD-dependent cytochrome P450 1A1 (CYP1A1) and CYP1B1 messenger RNA (mRNA) expression levels and recruitment of AHR to both genes. Overexpression of TiPARP reduced AHR-dependent increases in CYP1A1-reporter gene activity, which was restored by overexpression of AHR, but not aryl hydrocarbon receptor nuclear translocator. Deletion and mutagenesis studies showed that TiPARP-mediated inhibition of AHR required the zinc-finger and catalytic domains. TiPARP and AHR co-localized in the nucleus, directly interacted and both were recruited to CYP1A1 in response to TCDD. Overexpression of Tiparp enhanced, whereas RNAi-mediated knockdown of TiPARP reduced TCDD-dependent AHR proteolytic degradation. TCDD-dependent induction of AHR target genes was enhanced in Tiparp – / – mouse embryonic fibroblasts compared with wildtype controls. Our findings show that TiPARP is a mono-ADP-ribosyltransferase and a transcriptional repressor of AHR, revealing a novel negative feedback loop in AHR signalling.
    Print ISSN: 0305-1048
    Electronic ISSN: 1362-4962
    Topics: Biology
    Published by Oxford University Press
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  • 6
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    Airborne Cloud Detector (1985)
    In:  Other Sources
    Details
    Publication Date: 2019-06-28
    Description: Bonded to aircraft skin, it facilitates cloud avoidance. Airborne cloud detector consists of three major components: Aluminum patch durably bonded to aircraft skin, surge arrester, and dual-sensitivity charge-rate amplifier. Operation based on fact that aircraft surfaces become charged when ice or water particles strike them. Using increased gain sensitivities, cloudparticle detector reliable, noise-free, low-cost, high-sensitivity indicator of type of clouds that cause most problems for LFC aircraft at cruise altitude.
    Keywords: ELECTRONIC SYSTEMS
    Type: LAR-13137 , NASA Tech Briefs (ISSN 0145-319X); 9; 2; P. 63
    Format: text
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    NASA TECHNICAL REPORTS
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