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  • 1995-1999  (6)
  • 82.50  (2)
  • Key words Amyotrophic lateral sclerosis  (2)
  • sympathetic hyperactivity  (2)
  • cDNA cloning
  • 1
    Electronic Resource
    Electronic Resource
    Measurements of235U/238U isotopic ratio in the photoproduct UF5 by multiphoton ionization and time-of-flight mass spectrometry (1996)
    Springer
    Applied physics 62 (1996), S. 515-519 
    Details
    ISSN: 1432-0649
    Keywords: 07.75 ; 33.00 ; 82.50
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract A MultiPhoton-Ionization Time-Of-Flight Mass Spectrometry (MPI/TOFMS) apparatus was developed for real-time measurement of the uranium isotopic ratio in nascent UF5 formed by the 266 nm photolysis of effusive UF6 ( 〈 300 K, ≈ 1.3 × 10−4 Pa). The UF5 was selectively and efficiently multiphoton ionized by 532 nm radiation at appreciably low fluences ( 〈 10 J/cm2). The main ions observed, U+ and U2+, were subsequently analyzed with a TOFMS with mass resolution of 1190 to separate235U n+ and238U n+ completely. The isotopic ratio measurements showed good precision resulting from the excellent agreement which was observed between the isotopic ratios in UF5 products and those in a parent UF6 sample. These results suggested that the MPI/TOFMS method can be applied to the real-time analysis of separation factors in the molecular laser isotope separation of uranium by ionization of UF5 following the infrared photodissociation of UF6.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01081053
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Contrast ratio and separation factor in multiphoton dissociation of supercooled UF6 irradiated with multifrequency 16 μm Raman-laser radiation (1996)
    Springer
    Applied physics 62 (1996), S. 77-81 
    Details
    ISSN: 1432-0649
    Keywords: 82.50 ; 33.00
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract In infrared multiphoton dissociation of supercooled UF6 irradiated with multifrequency para-H2 Raman laser beams, separation factors and contrast ratios were measured by selective multiphoton ionization of the photoproduct UF5 at 532 nm followed by time-of-flight mass spectrometric analysis. The relationship between separation factor and contrast ratio is discussed theoretically and quantitatively investigated in the experiments. From this relationship, we obtain the irradiation conditions of the laser-beam fluences for attaining a high separation factor and estimate the value of the intrinsic separation factor in multifrequency dissociation of UF6.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01081251
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Absence of SOD1 gene abnormalities in familial amyotrophic lateral sclerosis with posterior column involvement without Lewy-body-like hyaline inclusions (1996)
    Springer
    Acta neuropathologica 92 (1996), S. 528-533 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; SOD1 gene ; Posterior column ; Lewy-body-like inclusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 65-year-old man with familial amyotrophic lateral sclerosis (ALS) with posterior column involvement showed fairly slow progression of the illness and lived with the aid of a respirator for 12 years. Neuropathological examinations showed simultaneous involvement of the pyramidal tract and lower motor neurons as well as degeneration in the Clarke’s nucleus- spinocerebellar tract- middle root zone of the posterior column, the pallido-luysian system, the medullary reticular formation, and widespread anterolateral columns of the spinal cord. However, the patient had no Lewy-body-like hyaline inclusions, which are characteristic features of this form of familial ALS. Moreover, no abnormalities were found in his SOD1 cDNA sequences. There seem to be certain heterogeneities in familial ALS with posterior column involvement, and SOD1 gene abnormalities may be involved in the pathomechanism in rapidly progressing ALS, in which there are Lewy-body-like hyaline inclusions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050557
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Astrocytic hyaline inclusions contain advanced glycation endproducts in familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutation: immunohistochemical and immunoelectron microscopical analyses (1999)
    Springer
    Acta neuropathologica 97 (1999), S. 260-266 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Nɛ-Carboxymethyl lysine ; Advanced glycation ; endproducts ; Superoxide dismutase 1 ; Astrocytic ; hyaline inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To clarify the neuropathological significance of the deposition of N ɛ -carboxymethyl lysine (CML), an advanced glycation endproduct, in astrocytic hyaline inclusions in familial amyotrophic lateral sclerosis (FALS), autopsy specimens from five members of two different families who had the superoxide dismutase 1 (SOD1) gene mutations were analysed. Immunohistochemically, most of the neuronal and astrocytic hyaline inclusions were intensely stained by the antibody against CML. The distributions and intensities of the immunoreactivities for CML and SOD1 were similar in the inclusions in both cell types. Immunoelectron microscopy showed that both inclusions consisted of CML-positive granule-coated fibrils and granular materials. No significant CML or SOD1 immunoreactivity was observed in the neurons and astrocytes of the normal control subjects. Our results suggest that astrocytic hyaline inclusions contain CML and SOD1 in FALS patients with SOD1 gene mutations, and that the formation of CML-modified protein (probably CML-modified SOD1) is related to the cell degeneration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050983
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Hyposensitivity of peripheral α-adrenoceptors in respiratordependent amyotrophic lateral sclerosis assessed by intravenous norepinephrine infusion (1995)
    Springer
    Clinical autonomic research 5 (1995), S. 165-169 
    Details
    ISSN: 1619-1560
    Keywords: amyotrophic lateral sclerosis ; down-regulation ; adrenoceptors ; sympathetic hyperactivity ; circulatory collapse ; norepinephrine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Intravenous norepinephrine infusion test was performed in eight patients with amyotrophic lateral sclerosis (ALS) supported by respirators and nine control subjects, to examine α-adrenoceptor function of peripheral resistant blood vessels. Baseline plasma norepinephrine concentrations in ALS patients were significantly higher than those in control subjects, indicating basal sympathetic hyperactivity (normal 218.2 ± 59.7 pg/ml; ALS 450.0 ± 288.4 pg/ml). The stimulus-response curves in the patients were similar to those in control subjects, and there were no significant differences between mean gains of the stimulus—response curves in both groups (normal 18.7 ± 5.5; ALS 15.2 ± 11.2). However, three ALS patients, two of whom had circulatory fluctuation and sympathetic hyperactivity, revealed lower gain levels than the mean minus 2 SD in control subjects (4.7, 1.1 and 3.7). This indicates hyposensitivity or down-regulation of the α-adrenoceptor function of peripheral blood vessels in these ALS patients. For early detection of sympathetic hyperactivity and prediction of circulatory collapse, it would be useful to measure the plasma norepinephrine concentration and the gain of the norepinephrine infusion curve in respirator-dependent ALS patients.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01826200
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    Paper (German National Licenses)
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  • 6
    Electronic Resource
    Electronic Resource
    Amyotrophic lateral sclerosis with hypertensive attacks: Blood pressure changes in response to drug administration (1996)
    Springer
    Clinical autonomic research 6 (1996), S. 241-244 
    Details
    ISSN: 1619-1560
    Keywords: amyotrophic lateral sclerosis ; hypertensive attacks ; sympathetic hyperactivity ; phentolamine ; propranolol
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Blood pressure changes in response to intravenous drug administration were examined in a respirator-dependent 49-year-old patient with sporadic amyotrophic lateral sclerosis (ALS) who developed severe hypertensive attacks. She showed severe hypertension and tachycardia during the daytime and nocturnal hypotension without compensatory tachycardia, which were consistent with the autonomic phenomenon reported in ALS patients. Infusion of phentolamine (2.5 mg) induced an abrupt 90 mmHg decrease in systolic pressure and slight increase in heart rate. Propranolol (1 mg) infusion induced decreases in both systolic pressure (36 mmHg) and heart rate (17 beats/min), although the pressure decrease was transient while the heart rate remained at the decreased level. Infusion of diazepam (10 mg) induced a 47 mmHg decrease in systolic pressure and a 23 beats/min increase in heart rate. These vasomotor responses indicate the distinct participation of abnormally augmented sympathetic tone, and especially of α-sympathetic hyperactivity rather than of β-sympathetic hyperactivity, in the hypertensive attacks occurring in this ALS patient.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02291141
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    Paper (German National Licenses)
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