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)

Brainstem acoustic evoked potentials (BAEPs) were measured in 14 children with different type of posterior fossa tumours several times during the clinical course, in order to assess the value of this simple and non-invasive method in the diagnosis and follow-up of posterior fossa tumours in childhood. Eight children had midline medulloblastoma, three children had lateral astrocytoma, three had intrinsic brainstem glioma. Different BAEP patterns could be detected in different tumour's type: bilateral symmetrical or slightly asymmetrical I-V. IPL prolongation in midline medulloblastomas, unilateral or markedly asymmetrical I.-V. IPL prolongation or wave V. depression on the contralateral side in lateral astrocytomas, and severely distorted asymmetrical waveform in intrinsic brainstem gliomas. The BAEPs were abnormal earlier than CT scan in a case of craniospinal astrocytoma. BAEPs were useful in the follow-up: the effect of the preoperative chemotherapy or the progression of the inoperable tumours could be as well documented by this method, as by the CT scan. BAEPs proved effective in the assessment of postoperative neurological complications: bilateral symmetrical IPL prolongation and wave V. depression with clinical signs of increased intracranial pressure occurred in a case of postoperative occlusive hydrocephalus, unilateral IPL prolongation occurred during irradiation or chemotherapy after medulloblastoma removal as signs of cerebral oedema.
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PMID:Value of brainstem acoustic evoked potentials in posterior fossa tumours in childhood. 186 81

Brainstem acoustic evoked potentials (BAEPs) were recorded in 16 children with posterior fossa tumours. The results were compared with the clinical course, CT scan findings and the postmortem pathological findings. Eight children had midline medulloblastoma, four children had lateral astrocytoma (two of them cerebellar, one of them pontocerebellar, and one of them craniospinal) and four children had brainstem glioma. Different BAEP pattern could be detected according to the tumour's location and histological nature: 1. In midline medulloblastoma: bilateral, symmetrical, or slightly asymmetrical I-V. IPL prolongation was the common abnormality. Beside that, some of the patients showed III-V., or I-III. IPL prolongation, or V. depression. 2. In lateral astrocytoma: asymmetrical BAEP abnormalities were seen: unilateral V. depression, and/or I-V. IPL prolongation. 3. In brainstem glioma: severely distorted waveform could be observed, with depression and gradual disappearance of components following the wave III. Based on these results BAEP measurement seems to be useful in the differential diagnosis of posterior fossa tumours of childhood, and it can be necessary even beside the CT scan. It can be useful in the early diagnosis, because the BAEP positivity can precede the CT scan positivity, in such a case repeated CT scan required. Finally BAEP measurement proved to be effective in the follow-up of posterior fossa tumours: it can document the tumour's chemotherapeutic regression, or the progression. It is a simple, non-invasive, and cheap method.
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PMID:[Brainstem acoustic evoked potential (BEAP] in children with posterior fossa tumors]. 231 75

Light-evoked excitatory cation current (DeltaIC) and inhibitory chloride current (DeltaICl) of rod and cone bipolar cells and AII amacrine cells (AIIACs) were recorded from slices of dark-adapted mouse retinas, and alpha ganglion cells were recorded from flatmounts of dark-adapted mouse retinas. The cell morphology was revealed by Lucifer yellow fluorescence with a confocal microscope. DeltaIC of all rod depolarizing bipolar cells (DBCRs) exhibited similar high sensitivity to 500 nm light, but two patterns of DeltaICl were observed with slightly different axon morphologies. At least two types of cone depolarizing bipolar cells (DBCCs) were identified: one with axon terminals ramified in 70-85% of IPL depth and DBCR-like DeltaIC sensitivity, and the other with axon terminals ramified in 55-75% of IPL depth and much lower DeltaIC sensitivity. The relative rod/cone inputs to DBCs and AIIACs were analyzed by comparing the DeltaIC and DeltaICl thresholds and dynamic ranges with the corresponding values of rods and cones. On average, the sensitivity of a DBCR to the 500 nm light is about 20 times higher than that of a rod. The sensitivity of an AIIAC is more than 1000 times higher than that of a rod, suggesting that AIIAC responses are pooled through a coupled network of about 40 AIIACs. Interactions of rod and cone signals in dark-adapted mouse retinas appear asymmetrical: rod signals spread into the cone system more efficiently than cone signals into the rod system. The mouse synaptic circuitry allows small rod signals to be highly amplified and effectively transmitted to the cone system via rod/cone and AIIAC/DBCC coupling. Three types of alpha ganglion cells (alphaGCs) were identified. (1) ONGCs exhibits no spike activity in darkness, increased spikes in light, sustained inward DeltaIC, sustained outward DeltaICl of varying amplitude, and large soma (20-25 microm in diameter) with an alpha-cell-like dendritic field about 180-350 microm stratifying near 70% of the IPL depth. (2) Transient OFFalphaGCs (tOFFalphaGCs) exhibit no spike activity in darkness, transient increased spikes at light offset, small sustained outward DeltaIC in light, a large transient inward DeltaIC at light offset, a sustained outward DeltaICl, and a morphology similar to the ONalphaGCs except for that their dendrites stratified near 30% of the IPL depth. (3) Sustained OFFalpha GCs (sOFFalphaGCs) exhibit maintained spike activity of 5-10 Hz in darkness, sustained decrease of spikes in light, sustained outward DeltaIC, sustained outward DeltaICl, and a morphology similar to the tOFFalphaGCs. By comparing the response thresholds and dynamic ranges of alphaGCs with those of the pre-ganglion cells, our data suggest that the light responses of each type of alphaGCs are mediated by different sets of bipolar cells and amacrine cells.
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PMID:Synaptic circuitry mediating light-evoked signals in dark-adapted mouse retina. 1553 95

Pleckstrin homology-like domain family A member 2 (PHLDA2) is a maternally expressed imprinted gene whose elevated expression has been linked to fetal growth restriction in a number of human studies. In mice, Phlda2 negatively regulates placental growth and limits the accumulation of placental glycogen. We previously reported that a three-copy transgene spanning the Phlda2 locus drove a fetal growth restriction phenotype late in gestation, suggesting a causative role for PHLDA2 in human growth restriction. However, in this mouse model, Phlda2 was overexpressed by fourfold, alongside overexpression of a second imprinted gene, Slc22a18. Here, we genetically isolate the role of Phlda2 in driving late fetal growth restriction in mice. We furthermore show that this Phlda2-driven growth restriction is asymmetrical, with a relative sparing of the brain, followed by rapid catch-up growth after birth, classic features of placental insufficiency. Strikingly, fetal growth restriction showed strain-specific differences, being apparent on the 129S2/SvHsd (129) genetic background and absent on the C57BL6 (BL6) background. A key difference between these two strains is the placenta. Specifically, BL6 placentae possess a more extensive endocrine compartment and substantially greater stores of placental glycogen. Taken together, these data support a direct role for elevated Phlda2 in limiting fetal growth but also suggest that growth restriction only manifests when there is limited placental reserve. These findings should be taken into account in interpreting the results from human studies.
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PMID:Isolating the role of elevated Phlda2 in asymmetric late fetal growth restriction in mice. 2508 93

Genomic imprinting disorders often exhibit delayed neurobehavioral development, suggesting this unique mechanism of epigenetic regulation plays a role in mental and neurological health. While major errors in imprinting have been linked to adverse health outcomes, there has been little research conducted on how moderate variability in imprinted gene expression within a population contributes to differences in neurobehavioral outcomes, particularly at birth. Here, we profiled the expression of 108 known and putative imprinted genes in human placenta samples from 615 infants assessed by the Neonatal Intensive Care Unit (NICU) Network Neurobehavioral Scales (NNNS). Data reduction identified 10 genes (DLX5, DHCR24, VTRNA2-1, PHLDA2, NPAP1, FAM50B, GNAS-AS1, PAX8-AS1, SHANK2, and COPG2IT1) whose expression could distinguish between newborn neurobehavioral profiles derived from the NNNS. Clustering infants based on the expression pattern of these genes identified 2 groups of infants characterized by reduced quality of movement, increased signs of asymmetrical and non-optimal reflexes, and increased odds of demonstrating increased signs of physiologic stress and abstinence. Overall, these results suggest that common variation in placental imprinted gene expression is linked to suboptimal performance on scales of neurological functioning as well as with increased signs of physiologic stress, highlighting the central importance of the control of expression of these genes in the placenta for neurobehavioral development.
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PMID:Expression of imprinted genes in placenta is associated with infant neurobehavioral development. 2619 1