Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Vimentin immunoreactivity was examined in brain tissues from non-neurological and various human central nervous system disease cases. In all brain tissues examined, vimentin immunoreactivity was intensely positive in ependymal cells and subpial tissues, and weakly positive in some capillaries and some white matter astrocytes. In affected areas of Alzheimer's disease (AD), Pick's disease, amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS) and cerebral infarction cases, numerous intensely vimentin-immunopositive astrocytes of both protoplasmic and fibrous morphology were demonstrated. A few such astrocytes were also observed in Parkinson's disease and progressive supranuclear palsy. ALS, MS and infarction brains also had numerous, strongly vimentin-positive, round and fat-laden microglia/macrophages. In AD and ALS, a few reactive microglia with irregularly enlarged shapes were vimentin positive. In AD, they were almost exclusively related to senile plaques.
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PMID:Vimentin immunoreactivity in normal and pathological human brain tissue. 152 71

Many observations have been carried out on astrogliosis in the cerebral cortex in amyotrophic lateral sclerosis (ALS), whereas little attention has been paid to astrogliosis in the spinal cord. Twenty autopsy cases of sporadic, common form of ALS have been studied. Spinal cords have been examined at the cervical, thoracic and lumbar levels by histological methods and immunohistochemistry for GFAP, Vimentin, Tau-protein, Neurofilaments, PCNA. A gliosis was found in the ventral horns, in dorsal horns and at the transition between gray matter and anterior and lateral funiculi, especially close to laminae VII, VI and V as being due to secondary gliosis. The findings cannot be interpreted on the only basis of the substitutive role of reacting glia. The proposed pathogenetic mechanisms of ALS are evaluated as possible responsible stimuli; the coincidence of the distribution of reactive astrocytes with the entering points of the corticospinal tracts into the gray matter is considered of primary importance. Of special interest are reactive astrocytes at the transition between laminae VII, VI and V and the lateral funiculus, where dystrophic neurites are known to concentrate.
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PMID:Reactive astrogliosis of the spinal cord in amyotrophic lateral sclerosis. 889 54

Ubiquilin1 (UBQLN1) is a ubiquitin-like domain and a ubiquitin-associated domain containing protein that has been reported to be involved in shuttling proteins to the proteasome, especially during endoplasmic reticulum-associated protein degradation. Thus, UBQLN1 function has been shown to be critical for combating a number of neurological disorders caused by protein aggregation, such as amyotrophic lateral sclerosis, Alzheimer's disease and Huntington's disease. A role for UBQLN1 in regulating processes involved in tumorigenesis has not been demonstrated. Herein, we show that loss of UBQLN1 causes increased cell migration and invasion, actin cytoskeleton reorganization and induction of epithelial-to-mesenchymal transition (EMT). Loss of UBQLN1 results in a significant decrease in the expression of epithelial markers including E-cadherin and claudin1, whereas expression of mesenchymal markers including Vimentin, Snail and ZEB1 are significantly elevated. Interestingly, we found that ZEB1 is required for induction of mesenchymal-like properties following loss of UBQLN1 and ZEB1 is capable of repressing expression of UBQLN1, suggesting a physiological, reciprocal regulation of EMT by UBQLN1 and ZEB1. Further, we find evidence for a role for UBQLN2 in also regulating EMT and cell migration. These observations have potential clinical relevance because the UBQLN1 gene is lost and underexpressed in a large percentage of human cancer cell lines, and primary human lung cancer samples and recurrent mutations in all five UBQLN family members have been identified in human lung cancers. Taken together, our results suggest for the first time a role for UBQLN family members in cancer biology.
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PMID:Ubiquilin1 represses migration and epithelial-to-mesenchymal transition of human non-small cell lung cancer cells. 2474 70

Vimentin-containing cells (VCCs) are potential neural precursor cells in central nervous systems, Thus, we studied the alteration of VCCs proliferation, differentiation and migration in the cerebrum during different stages of Tg(SOD1*G93A)1Gur mice. It aims to search potential ways regulating the proliferation, differentiation and migration of endogenous VCCs, to enhance their neural repair function and to cure or prevent from the development of ALS. We observed and analyzed the proliferation, differentiation and migration of VCCs in different anatomic regions and cell types of cerebrum at different stages including the pre-onset (60-70 days), onset (90-100 days) and progression (120-130 days) of wild-type (WT) and Tg(SOD1*G93A)1Gur mice using the fluorescent immunohistochemical technology. Results showed that VCCs in the cerebrum were mostly distributed in the ventricular system, periventricular structures, the hippocampus and the cerebral cortex in WT mice. VCCs significantly reduced in the motor cortex and the cingulate cortex in Tg(SOD1*G93A)1Gur mice. All vimentin expressed in the extranuclear and almost all VCCs were astrocytes in WT mice and Tg(SOD1*G93A)1Gur mice. There were no significant difference in the number of Brdu and nestin positive cells in left and right brains of WT mice and Tg(SOD1*G93A)1Gur mice in the period of 60-130 days. Our data suggested that there existed extensively NPCs in the cerebrum of adult mice. In ALS-like Tg(SOD1*G93A)1Gur mice, VCCs in the motor cortex, the olfactory cortex and the cingulate cortex showed that no any proliferation and redistribution in neural cells of VCCs in the cerebrum occurred in all stages of ALS, might migrate to damaged regions.
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PMID:Altered Features of Vimentin-containing Cells in Cerebrum of Tg(SOD1*G93A)1Gur Mice: A Preliminary Study on Cerebrum Endogenous Neural Precursor Cells in Amyotrophic Lateral Sclerosis. 3185 21