Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Interaural latency differences (ILDs) of wave V of the brainstem electric response (BSER) were studied (1) in 20 normal-hearing subjects at 90, 80, 60 and 40 dB HL click level, (2) in 22 patients with symmetrical cochlear hearing loss at 90 dB HL, corresponding to 45-80 dB SL, and (3) in 45 patients with asymetrical cochlear hearing loss with interaural recruitment, at 90 dB HL on one hand and at a click sensation level corresponding to 90 dB HL in the poorer ear (range 10-75 dB SL) on the other hand. In the normal hearing group the ILDs did not exceed 0.2 ms except in one case at 40 dB HL (0.3 ms). In the symmetrical cochlear loss group the ILD did not exceed 0.2 ms in any patient. In the asymmetrical cochlear loss group the ILD at 90 dB HL exceeded 0.2 ms in 12 cases, but only in four cases after correction for pure tone hearing loss at 4 kHz by 0.1 for each 10 dB above 50 dB HL; at equal sensation levels the ILDs were all zero or negative meaning that the wave V latency was shorter on stimulation of the poorer ear. Assuming ILD to be a valid discriminator between cochlear and retrocochlear lesions, the corrected ILD at equal click hearing levels (90 dB HL), with the critical value set at 0.2 ms, thus gave a 9% false-positive rate whereas the ILS at equal sensation levels, with the critical value set at zero, gave no false-positives. It still remains to be shown that the latter criterion will not increase the number of false-negative results in retrocochlear lesions.
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PMID:On the use of click-evoked electric brainstem responses in audiological diagnosis. IV. Interaural latency differences (wave V) in cochlear hearing loss. 728 May 43

Neurons in the cerebral cortex originate predominantly from asymmetrical divisions of polarized radial glial or neuroepithelial cells. Fate control of neural progenitors through regulating cell division asymmetry determines the final cortical neuronal number and organization. Haploinsufficiency of human LIS1 results in type I lissencephaly (smooth brain) with severely reduced surface area and laminar organization of the cerebral cortex. Here we show that LIS1 and its binding protein Nde1 (mNudE) regulate the fate of radial glial progenitors collaboratively. Mice with an allelic series of Lis1 and Nde1 double mutations displayed a striking dose-dependent size reduction and de-lamination of the cerebral cortex. The neocortex of the Lis1-Nde1 double mutant mice showed over 80% reduction in surface area and inverted neuronal layers. Dramatically increased neuronal differentiation at the onset of corticogenesis in the mutant led to overproduction and abnormal development of earliest-born preplate neurons and Cajal-Retzius cells at the expense of progenitors. While both Lis1 and Nde1 are known to regulate the mitotic spindle orientation, only a moderate alteration in mitotic cleavage orientation was detected in the Lis1-Nde1 double deficient progenitors. Instead, a striking change in the morphology of metaphase progenitors with reduced apical attachment to the ventricular surface and weakened lateral contacts to neighboring cells appear to hinder the accurate control of cell division asymmetry and underlie the dramatically increased neuronal differentiation. Our data suggest that maintaining the shape and cell-cell interactions of radial glial neuroepithelial progenitors by the Lis1-Nde1 complex is essential for their self renewal during the early phase of corticogenesis.
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PMID:Lis1-Nde1-dependent neuronal fate control determines cerebral cortical size and lamination. 1846 43

The fine tuning of proliferation and neurogenesis, neuronal migration and differentiation and connectivity underlies the proper development of the cerebral cortex. Mutations in genes involved in these processes are responsible for neurodevelopmental disorders, such as cortical dysgeneses, which are usually associated with severe mental retardation and epilepsy. Over the past few years, the importance of cytoskeleton components in cellular processes crucial for cortical development has emerged from a body of functional data. This was reinforced by the association of mutations in the LIS1 and DCX genes, which both encode proteins involved in microtubule (MT) homeostasis, with cerebral cortex developmental disorders. The recent discovery of patients with lissencephaly and bilateral asymmetrical polymicrogyria (PMG) carrying mutations in the alpha- and beta-tubulin-encoding genes TUBA1A and TUBB2B further supports this view, and also raises interesting questions about the specific roles played by certain tubulin isotypes during the development of the cortex.
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PMID:Tubulin-related cortical dysgeneses: microtubule dysfunction underlying neuronal migration defects. 1986 38