1,916 research outputs found
Efficient operation of a solid-state adaptive laser oscillator
No full textWe present the results of a cw diode-pumped Nd:YVO4 laser oscillator based on a self-starting adaptive gain-grating resonator. Adaptive laser operation has been demonstrated with 12-W output for 37 W of diode pumping, producing a TEM00 mode that compensates for thermal aberrations. The issue of the finite aperture of the amplifier is discussed, and a design that incorporates an intracavity lens is used to improve the collection efficiency with severe thermal lensing at high pump powers. The powers of the beams involved in the resonator are compared with theory and are found to be in good agreement. Spectral and temporal behavior of the adaptive laser is investigated, and very interesting behavior is shown, including self-induced temporal modulation dynamics and a switching between a narrowband and a broad bandwidth of operation
Taxa of Coleoptera described by M.E. Ter-Minassian
No full textMargarita Ervandovna Ter-Minassian was an outstanding expert in the taxonomy of the Coleoptera, primarily of the family Bruchidae and superfamily Curculionoidea. She has described 8 genera, 3 subgenera and more than 180 species. The full list of the taxa described by her is given in alphabetic order, with indication of their position in the modern classification. Apion terminasianae Alonso-Zarazaga, 1986 is returned to the genus Apion Herbst, 1797 from Kalcapion Schilsky, 1906
Power-scaling continuous-wave adaptive laser resonators
No full textWe demonstrate a power-scaling strategy in a continuous-wave adaptive laser resonator that actively corrects, via phase-conjugation, for thermally induced phase distortions introduced by a power-amplifier placed in the output arm of the resonator
Efficient adaptive self-starting Nd:YVO<sub>4</sub> gain grating laser oscillator
No full textWe present the results of a self-starting continuous-wave diode-pumped Nd:WO4 laser oscillator based on an adaptive intracavity gain-grating technique. The laser produces 12W narrowband output in a TEMoo mode with 36W of diode-pumping
Progressive myoclonus epilepsy with polyglucosans (Lafora disease): evidence for a third locus
No full textLafora disease (LD) is the most common teenage-onset progressive myoclonus epilepsy. It is caused by recessive mutations in the EPM2A or EPM2B genes. The authors describe a family with three affected members with no mutations in either gene. Linkage and haplotype analyses exclude both loci from causative involvement in this family. Therefore, a third LD locus is predicted. Its identification will be a crucial element in the understanding of the biochemical pathway underlying the generation of Lafora bodies and LD
Survey report 1978/79 Glaciology department Author - B.A. Murphy
No full textProgress Code: completedStatement: See the report for further information.
The values provided in spatial coverage are approximate only. The values provided in temporal coverage are based on the assumption that B.A. Murphy travelled to and from Davis by scheduled Australian Antarctic voyages.Taken from sections of the report:<br/><br/>Introduction:<br/><br/>The following report is a detailed summary of the surveying and mapping tasks undertaken in the Vestfold Hills and Mac. Robertson Land regions of the Australian Antarctic Territory during the period from 22 December 1978 to 25 February 1979. A copy of the project instruction detailing the tasks originally intended to be undertaken is attached at Annex 37.<br/><br/>The entire report is available as a pdf download from the URL given below
Genetic diagnosis in Lafora disease: Genotype-phenotype correlations and diagnostic pitfalls
No full textLafora disease (LD) can be diagnosed by skin biopsy, but this approach has both false negatives and false positives. Biopsies of other organs can also be diagnostic but are more invasive. Genetic diagnosis is also possible but can be inconclusive, for example, in patients with only one heterozygous EPM2A mutation and patients with apparently homozygous EPM2B mutations where one parent is not a carrier of the mutation. We sought to identify occult mutations and clarify the genotypes and confirm the diagnosis of LD in patients with apparent nonrecessive disease inheritance. We used single nucleotide polymorphism, quantitative PCR, and fluorescent in situ hybridization analyses. We identified large EPM2A and EPM2B deletions undetectable by PCR in the heterozygous state and describe simple methods for their routine detection. We report a coding sequence change in several patients and describe why the pathogenic role of this change remains unclear. We confirm that adult-onset LD is due to EPM2B mutations. Finally, we report major intrafamilial heterogeneity in age at onset in LD. ©2007AAN Enterprises, Inc.Andrade DM, 2003, NEUROLOGY, V61, P1611; Baykan B, 2005, EPILEPSIA, V46, P1695, DOI 10.1111-j.1528-1167.2005.00272.x; BUSARD HLSM, 1987, ANN NEUROL, V21, P599, DOI 10.1002-ana.410210613; CARPENTER S, 1981, ANN NEUROL, V10, P63, DOI 10.1002-ana.410100116; Chan EM, 2004, NEUROLOGY, V63, P565; Chan EM, 2003, NAT GENET, V35, P125, DOI 10.1038-ng1238; Chan EM, 2004, HUM MOL GENET, V13, P1117, DOI 10.1093-hmg-ddh130; Fernandez-Barreiro A, 1999, J NEUROL NEUROSUR PS, V66, P114; Fernandez-Sanchez ME, 2003, HUM MOL GENET, V12, P3161, DOI 10.1093-hmg-ddg340; Footitt DR, 1997, J NEUROL, V244, P40; Franceschetti S, 2006, EPILEPSIA, V47, P640, DOI 10.1111-j.1528-1167.2006.00479.x; Ganesh S, 2002, HUM MOL GENET, V11, P1263, DOI 10.1093-hmg-11.11.1263; Ganesh S, 2004, BIOCHEM BIOPH RES CO, V313, P1101, DOI 10.1016-j.bbrc.2003.12.043; Ganesh S, 2000, HUM MOL GENET, V9, P2251; Gentry MS, 2005, P NATL ACAD SCI USA, V102, P8501, DOI 10.1073-pnas.0503285102; Gomez-Abad C, 2005, NEUROLOGY, V64, P982; Gomez-Garre P, 2000, EUR J HUM GENET, V8, P946, DOI 10.1038-sj.ejhg.5200571; Ianzano Leonarda, 2005, Hum Mutat, V26, P397, DOI 10.1002-humu.9376; KAUFMAN MA, 1993, NEUROLOGY, V43, P1246; Lafora GR, 1911, Z GESAMTE NEUROL PSY, V6, P1, DOI 10.1007-BF02863929; Lohi H, 2005, HUM MOL GENET, V14, P2727, DOI 10.1093-hmg-ddi306; Lohi Hannes, 2006, Adv Neurol, V97, P399; Messouak O, 2002, REV NEUROL-FRANCE, V158, P74; Minassian BA, 1998, NAT GENET, V20, P171, DOI 10.1038-2470; Minassian BA, 2001, PEDIATR NEUROL, V25, P21, DOI 10.1016-S0887-8994(00)00276-9; Minassian BA, 2000, NEUROLOGY, V55, P341; Minassian BA, 2000, NEUROLOGY, V54, P488; Singh S, 2005, J HUM GENET, V50, P347, DOI 10.1007-s10038-005-0263-7; Van Heycop Ten Ham MV, 1974, HDB CLIN NEUROLOGY, V15, P382; Wang JY, 2002, J BIOL CHEM, V277, P2377, DOI 10.1074-jbc.C100686200; Wang W, 2004, BIOCHEM BIOPH RES CO, V325, P726, DOI 10.1016-j.bbrc.2004.10.08321211
A SPONTANEOUS MOUSE MODEL OF X-LINKED MYOPATHY WITH EXCESSIVE AUTOPHAGY
No full textthe subject of the thesis research project, aims to characterize a murine model for spontaneous muscle pathology comparable to human "X-Linked Vacuolar myopathy with Excessive Autophagy (XMEA)". Along with Danon disease, these myopathies are characterized by the accumulation of vacuoles within the myofiber.
In 1988, Kalimo et al. have described five cases (all males) who had progressive proximal myopathy, which did not involve the heart muscle. The muscle biopsies of all patients showed numerous vacuoles, sarcoplasmic and subsarcolemmal, many of which were positive for the lysosomal enzyme, for the morphological appearance described, was proposed the term: X-Linked Vacuolar myopathy with Excessive Autophagy (XMEA).
We used crosses of strain C57/BL6 mice of different ages and sex, we processed triceps muscles, quadriceps femoris and the cranial tibial muscle and made morphological, histochemical and immunohistochemical staining on this samples. From laboratory tests performed on muscle biopsies, we found the presence of numerous vacuoles within muscle fibers, only in males. All these characters are comparable to XMEA.
The availability of a well characterized mouse model it may help define the etiopathogenesis of this disease
- …
