Search results for "EBE"
showing 10 items of 929 documents
Analysis of BNIP3 and BNIP3L/Nix expression in cybrid cell lines harboring two LHON-associated mutations.
2019
Mitochondria are key players in cell death through the activation of the intrinsic apoptosis pathway. BNIP3 and BNIP3L/Nix are outer mitochondrial membrane bifunctional proteins which because of containing both BH3 and LIR domains play a role in cellular response to stress by regulation of apoptosis and selective autophagy. Leber’s Hereditary Optic Neuropathy (LHON) is the most common mitochondrial disease in adults, characterized by painless loss of vision caused by atrophy of the optic nerve. The disease in over 90% of cases is caused by one of three mutations in the mitochondrial genome: 11778G>A, 3460G>A or 14484T>C. The pathogenic processes leading to optic nerve degeneration …
Simulating Images Seen by Patients with Inhomogeneous Sensitivity Losses
2012
PURPOSE We aim to simulate how colored images are perceived by subjects with local achromatic and chromatic contrast sensitivity losses in the visual field (VF). METHODS The spatiochromatic corresponding pair algorithm, introduced in a previous article (J Opt Soc Am (A) 2004;21:176-186), has been implemented with a linear model of the visual system. Spatial information is processed separately by the chromatic and achromatic mechanisms by means of a multiscale model, with sensors selective to frequency, orientation, and spatial position, whose mechanism-dependent relative weights change with the spatial location of the image. These weights have been obtained from perimetric data from a patie…
Cerebral and spinal MR-findings in patients with postrepair myelomeningocele
1990
In 114 patients with postrepair myelomeningocele MRI of the spine was performed. Tethered cord (89%) and associated malformations (syrinx, lipoma etc.) (33%) were the most important findings. Additional MRI scans of the head (44 patients) revealed numerous further anomalies. Arnold Chiari malformation was found in 76% of the patients (ACM I: 32%, ACM II: 44%). In the ACM II group compression of lower cranial nerves, brain stem, and cerebellum can lead to considerable neurologic symptoms. Therefore in patients with progressive neurologic dysfunction a complete investigation of the whole spine and brain is necessary. MRI proves to be the diagnostic procedure of choice in patients with dysraph…
A novel L1CAM mutation in a fetus detected by prenatal diagnosis
2010
X-linked hydrocephalus is due to mutations in the L1 neuronal cell adhesion molecule (L1CAM) gene. L1 protein plays a key role in neurite outgrowth, axonal guidance, and pathfinding during the development of the nervous system. We report on a familial case diagnosed by prenatal ultrasonographic examination, with cerebellar hypoplasia, agenesis of the corpus callosum, and the bilateral overlapping of the second and third fingers of the hand. Sequencing of the L1CAM gene showed a novel missense mutation in exon 14: transition of a guanine to cytosine at position 1777 (c.1777G > C), which led to an amino acid change of alanine to proline at position 593 (Ala593Pro) in the sixth immunoglobulin …
Pharmacological heterogeneity of γ-aminobutyric acid receptors during development suggests distinct classes of rat cerebellar granule cells in situ
2001
The gamma-aminobutyric acid receptor (GABA(A)R) represents a ligand-gated Cl(-)-channel assembling as heteropentamere from 19 known subunits. Cerebellar granule cells contain a unique subset, namely the alpha1-, alpha6-, beta2-, gamma2- and delta-subunits. We studied their GABAergic pharmacology in situ using whole-cell patch-clamp recordings in brain slices and a modified Y-tube application system. The distribution of the EC50s for GABA in young (P8-P14) and medium aged animals (P15-P28) could be fitted with the sum of two Gaussian distributions with means of 60 and 185 microM and 27 and 214 microM, respectively. In older animals (P29-P48) the observed homogeneous range of sensitivities fi…
Randomized, double-blind, placebo controlled, multicentre study of idebenone in patients suffering from multi-infarct dementia
1992
Abstract In this randomized double-blind, placebo controlled, multicentre study on 108 elderly patients with mild to moderate mental deterioration of vascular origin, idebenone — a benzoquinone derivative with a hydroxyalkyl side chain — proved to be therapeutically effective in the treatment of multi-infarct dementia. The oral administration of idebenone 45 mg/day b.i.d. for 120 days significantly improved the scores of the following test in comparison with placebo: Mini Mental State, Randt Memory Test, Gottfries Rating Scale, Token Test, Toulouse Pieron Test, indicating improvements in memory attention and cognitivity. The drug was well tolerated and effective in patients with multi-infar…
Idebenone in senile dementia of Alzheimer type: A multicentre study
1992
Idebenone is a new cerebro-active drug, effective in dementia disorders, particularly indicated in primary degenerative dementias, i.e. Alzheimer's disease. This new molecule acts as an electron trapper and a free radical scavenger protecting mitochondrial membranes from lipid peroxidation. A multicentric, double-blind trial of idebenone (45 mg twice daily orally) vs. placebo was carried out on 102 elderly patients affected by Alzheimer-type dementia of mild or moderate severity. Idebenone was administered for 4 consecutive months, 45 mg twice daily. Clinical evaluations were performed at the time of enrollment (t0) and monthly thereafter (t30, t60, t90 and t120) and at follow-up (t150 ). T…
Die landwirthschaftlich-chemische Versuchsstation am Polytechnikum zu Riga, Lief. I
1875
Bericht über die Thätigkeit in den Jahren 1872/73 und 1873/74
Somatosensory event-related potentials in the rabbit cerebral and cerebellar cortices: a correspondence with mismatch responses in humans.
2001
Somatosensory event-related potentials (ERPs) were recorded from the cerebellar (CerCx), somatosensory (SomCx) and visual (VCx) cortices in rabbits in two stimulus conditions. In the oddball condition, airpuffs to two different locations in the rabbit's muzzle corresponded to infrequently presented deviant stimuli (oddball-deviants) interspersed with frequently presented standard stimuli. In the deviant-alone condition, deviants (alone-deviants) were presented without standards. ERPs to oddball-deviants differed significantly from those to standards in CerCx and SomCx, but not in VCx. Furthermore, some of these differences were not found between ERPs to alone-deviants and those to standards…
Zvaigžņotā Debess: 2017, Pavasaris (235)
2017
Contents: “ZVAIGŽŅOTĀ DEBESS” FORTY YEARS AGO: E.Mūkins. Clouds of Venus: Structure and Composition (abridged) ; J.Miezis. 3rd All-union Young Astronomers Gathering (abridged) ; DEVELOPMENTS in SCIENCE: K.Schwartz. New Discoveries in the Milky Way ; DISCOVERIES: I.Eglītis. Small Planets Get Latvian Names ; I.Pundure. Earth-mass World Revealed around Proxima Centauri ; SPACE RESEARCH and EXPLORATION: J.Jaunbergs. Mysteries of Saturn’s Clouds ; A.Slavinskis. Contribution of Estonian Student Satellite Programme ESTCube to Largest Challenges for Humanity ; R.Misa. Impossible Engine that Is likely Possible ; I.Pundure. ESO VLT to Search for Planets in Alpha Centauri System ; EARTH and COSMOS: J.…