Esterification of all-trans-retinol is a key reaction of the vertebrate visual cycle, since it produces an insoluble, relatively non-toxic, form of the vitamin for storage and supplies substrate for the isomerization reaction. CoA-dependent and -independent pathways have been described for retinol esterification in retinal pigment epithelium (RPE). The CoA-independent reaction, catalysed by lecithin:retinol acyltransferase (LRAT) was examined in more detail in this study. Addition of retinol to RPE microsomes results in a burst of retinyl ester synthesis, followed by a rapid apparent cessation of the reaction. However, [3H]retinol, added when retinyl ester synthesis has apparently ceased, is rapidly incorporated into retinyl ester without a net increase in the amount of ester. The specific radioactivities of [3H]retinol and [3H]retinyl ester reach the same value. [14C]Palmitate from palmitoyl-CoA is incorporated into preexisting retinyl ester in the absence of net ester synthesis, too. These exchange reactions suggest that the reaction has reached equilibrium at the plateau of the progress curve and that only the accumulation of retinyl ester, and not its synthesis, has stopped during this phase of the reaction. Studies with geometrical isomers of retinol revealed that the rate of exchange of all-trans-retinol with all-trans-retinyl esters was about 6 times more rapid than exchange of 11-cis-retinol with 11-cis-retinyl ester. This is the first demonstration of the reversibility of LRAT and the first example of stereospecificity of retinyl ester synthesis in the visual system. Reversal of the LRAT reaction could contribute to the mobilization of 11-cis-retinol from 11-cis-retinyl ester pools.
A juvenile Siamese cat with severe, progressive motor disability was shown to have extensive neuronal degeneration caused by accumulation of GM(1) ganglioside. Tissues from brain and kidney were markedly deficient in beta-galactosidase activity. The disease in this cat is thought to be inherited as an autosomal recessive trait, and is strikingly similar to juvenile GM(1) gangliosidosis of children.
Summary:Purpose: Numerous factors have been analyzed in attempts to predict the outcome of surgical resections in patients with neocortical epilepsy. We examined the correlation between surgical outcome and electrocorticographic features of neocortical ictal patterns.Methods: Twenty six patients with neocortical epilepsy underwent monitoring with subdural grid electrodes before surgery. Ictal patterns were analyzed retrospectively and correlated with three types of outcome: seizure free, worthwhile improvement (>75% reduction of seizure frequency), and no worthwhile improvement. The duration of follow-up was 2-5 years.Results: lctal patterns were divided according to the size of epileptogenic zone (focal, regional, multifocal); velocity and type of seizure propagation (fast contiguous, slow contiguous, noncontiguous); pattern of the onset of ictal activity; part of the cortex involved in the origin of the seizure (frontal, frontocentroparietal, etc.). Spread to medial temporal structures (as assessed by subtemporal strips) also was evaluated in selected cases. Statistically significant correlation with surgical outcome (p = 0.026) was shown for only one variable: type of spread. Patients with slow spread (n = 8) demonstrated the best outcomes (five are seizure free), whereas patients with noncontiguous spread (n = 5 ) demonstrated the worst outcomes (four did not improve significantly). Patients with fast contiguous spread (n = 13) showed intermediate outcomes.Conclusions: Types of propagation of ictal neocortical activity correlate with surgical outcome. Analysis of ictal pattern during intracranial recordings may help to predict surgical outcome for neocortical epilepsy. Key Words: Ictal patternSeizure spread-Epileptogenic zone.Neocortical epilepsy presents a substantial problem from both medical and surgical perspectives. Medical management of neocortical epilepsy is difficult because of notoriously poor response to anticonvulsant medications (AEDs) and disconcertingly high frequency of seizures (1-3). Surgical management is challenging because of the elusive nature of ictal onset, which frequently requires intracranial monitoring ( 4 3 , and the frequently encountered dilemma of separating resectable epileptogenic regions from vital eloquent cortex (6).The outcome of epilepsy surgery for different types of neocortical epilepsy is substantially worse than that for medial temporal epilepsy. The variability in the seizurefree outcome rate is striking, ranging from 10% for selected posterior temporal, temporoparietal, and occipital cases (7) to 26% in a large retrospective review of Montreal Neurological Institute surgical cases (S), 43% in a combined multicenter study (9) to 53.7% in retrospective analysis performed at the Institute of Neurosurgery,
Purpose:The purpose of this paper is to report 14 new cases of unilateral retinitis pigmentosa and three new cases of cone-rod dystrophy and to compare the similarities and dissimilarities to those found in the bilateral forms of these disorders.Methods:A total of 272 cases of retinitis pigmentosa and 167 cases of cone-rod dystrophy were studied by corneal full field electroretinograms and electrooculograms. The student t-test was used to compare categories.Results:The percentage of familial and nonfamilial cases was the same for the bilateral and unilateral forms of the disease. In our series, unilateral retinitis pigmentosa makes up approximately 5% of the total population of retinitis pigmentosa, while unilateral cone-rod dystrophy makes up only about 2% of the total. In the familial forms of unilateral retinitis pigmentosa the most common inheritance pattern was autosomal dominant and all affected relatives had bilateral disease.Conclusion:Unilateral retinitis pigmentosa and cone-rod dystrophy appear to be directly related to the more common bilateral forms of these disorders. The genetic mechanisms which account for asymmetric disorders are not currently understood. It may be a different unidentified mutation at a single loci or it is possible that nonlinked mutations in multiple loci account for this unusual disorder.
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