Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: HUMANGGP:036206 (endoplasmic reticulum)
 63,868 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Light and electron microscopic studies were made on the anterior horn cells in a case o amyotrophic lateral sclerosis. Eosinophilic inclusions of Bunina type were observed almost selectively in the motor neurons of spinal cord, as well as of brain stem, at the light microscopic level. Fine structural study revealed the presence of two types of cytoplasmic inclusions. The first, mainly corresponding to the light microscopic inclusions, were homogeneous, electron-dense, round- or oval-shaped bodies with vesicular or tubular rims and ribosome particles, about 2-5 mu in diameter, which contained filaments or other cytoplasmic componenets in the clear areas within them. The second were lamellar structures (laminated cytoplasmic bodies, Morales) which appeared to be originating from endoplasmic reticulum. There was no distinct transition in these two types of inclusions and the relationship to each other is not clear. The significance of Bunina body is unknown, but some manifestation of a primary disorder, e.g., protein metabolism, rather than a secondary degenerative change in the motor neurons in amyotorophic lateral sclerosis.
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PMID:Ultrastructure of the Bunina bodies in anterior horn cells of amyotrophic lateral sclerosis. 65 89

A patient suffered recurrent episodes of aseptic lymphocytic meningitis for many years and then developed amyotrophic lateral sclerosis (ALS). Immune-complexes were deposited in the renal glomerular basement membrane and mesangia. The necropsy study revealed both lymphocytic meningitis and ALS. Study of the motor neurons with the electron microscope revealed proliferation of endoplasmic reticulum, small cytoplasmolytic areas and focal neurofibrillar accumulations in axons. Interwoven, serpentine 10-15 nm. tubules first appeared with ER proliferation and, presumably at a later stage, were sometimes present in large masses. These tubules might be virus material but virus cultures, including tissue culture, and animal inoculations have thus far been negative.
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PMID:Tubular particles in a case of recurrent lymphocytic meningitis followed by amyotrophic lateral sclerosis. 112 48

Recent decline in incidence rates of ALS in Guam and the Kii Peninsula of Japan strongly implicates environmental factors rather than inheritance in its causation. Environmental studies in Western Pacific foci showed identical mineral compositions in the soils and drinking water, i.e., extremely low calcium (Ca) and magnesium (Mg) and high aluminum (Al) and manganese (Mn). Series of trace-elemental analyses of the CNS tissue of ALS patients have revealed a high contents of Al and Ca with significant positive correlations between Al and Ca and/or between Ca and Mn, suggesting the prolonged exposure to these trace environment to cause abnormal mineral metabolism detrimental to neurons. Using electron energy loss spectrometry (EELS), Al was found to accumulate within DNA-containing chromatins and rRNA-containing cellular components, i.e., nucleolus, heterochromatin, rough endoplasmic reticulum, in lumbar motor neurons of ALS. Thus, Al may preferentially bind to nucleic acids and cause a progressive inhibition of the protein synthesis of rRNA and the transcription or gene modulation of DNA, leading to neuronal degeneration.
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PMID:[Environmental factors in western Pacific foci of ALS and a possible pathogenetic role of aluminum (Al) in motor neuron degeneration]. 181 96

Several senile changes in the central nervous system in cadavers were examined. The pattern of extension of Alzheimer's neurofibrillary tangles (NFT) and senile plaques (SP) in the olfactory bulbs of 100 specimens was examined during routine autopsy by immunohistochemical staining. NFT were first observed in the anterior olfactory nucleus after the age of 60, and incidence rose with increasing age. Senile plaques were found in the nucleus when there were many SP in the cerebral cortex. Of 25 non-demented amyotrophic lateral sclerosis patients, SP were found in the cerebral cortices of 10, and 9 of 10 were over 60 years old. NFT were observed in almost all patients over 60 years of age, but the incidence was low. Many ubiquitin-positive small-sized granules were observed in the second and third layer of the parahippocampal gyrus of aged patients, and the incidence rose with increasing age. On the other hand, few of these granules were in patients with Alzheimer's type dementia. Granulovacuolar degeneration was examined. Many centrally-located granules were positive for ubiquitin. Based on electron microscopic observation of these granules at several stages, the granules were thought to be a type of autophagosome. During the first stage of granulovacuolar degeneration, electron-dense materials appeared in the cytoplasm, following which they were surrounded by smooth endoplasmic reticulum. Analytical electron microscopy disclosed that the granules contained some aluminium.
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PMID:[Neuropathological aspects of normal and abnormal aging]. 189 21

The spinal cord and motor cortex of patients with amyotrophic lateral sclerosis (ALS) were examined with immunohistochemical methods for the presence of IgG. In 13 of 15 spinal cords, a population of motoneurons stained positively for IgG in a granular pattern, characteristic of binding to the rough endoplasmic reticulum. In 6 of 11 motor cortices, a proportion of pyramidal cells also stained positively for IgG. No such reactivity was noted in motoneurons of control human tissues, although positive IgG staining was present in astrocytes of ALS and control specimens. Reactive microglia and/or macrophages were detected in the territory of degenerating pyramidal tracts and ventral horns. The surface of most of these cells stained positively for IgG, and 50% stained positively for HLA-DR. The accumulation of IgG in motoneurons and the presence of immunologically active macrophages provide additional evidence for the participation of immunologic factors in the pathogenesis of ALS.
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PMID:IgG reactivity in the spinal cord and motor cortex in amyotrophic lateral sclerosis. 212 77

Ultrastructural features of chromatolytic neurons observed in a sporadic case with amyotrophic lateral sclerosis (ALS) are reported. A 70-year-old woman died of weakness and atrophy of the four limbs, bulbar and facial muscles, and hyperreflexia, of 3 1/2 years' duration. Neuronal loss was marked in the anterior horn of the spinal cord, with degeneration of the pyramidal tracts. Most of the remaining neurons showed chromatolysis. Some of the chromatolytic neurons contained faintly eosinophilic inclusions with a halo. Few spheroids were observed. Hypoglossal nuclei, nucleus ambiguus, motor nuclei of N.VII and N.V were well populated, but contained several chromatolytic neurons. Ultrastructurally, the chromatolytic neurons contained aggregates of fibrils thicker than the 10-nm neurofilaments. These fibrils were arranged randomly, and were closely associated with granular materials as well as rough endoplasmic reticulum. Neurofilamentous accumulations reported to be common in sporadic ALS were rare in this case. No Bunina body was observed.
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PMID:Ultrastructural study of chromatolytic neurons in an adult-onset sporadic case of amyotrophic lateral sclerosis. 337 56

Ultrastructural changes of hepatocytes were studied in liver biopsy specimens obtained from 21 patients with amyotrophic lateral sclerosis (ALS). In all specimens, one or more of the following abnormal findings were made: bizarre giant mitochondria; intramitochondrial paracrystalline inclusions; disorganization of the lamellar structure of rough endoplasmic reticulum; increased numbers of smooth endoplasmic reticulum; and/or parasinusoidal fibrosis. Above all, paracrystalline inclusions appeared to be a highly specific finding in ALS. In light of the above pathologic findings, we evaluated the liver function tests of 37 patients (including the above-mentioned 21 patients) with ALS, and found a high incidence of mild liver dysfunction. In spite of its subtleness, these ultrastructural changes of hepatocytes were consistently found in such patients, and offer further insight into the intrahepatic metabolic abnormalities in ALS.
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PMID:Hepatic ultrastructural changes and liver dysfunction in amyotrophic lateral sclerosis. 380 Jul 8

In an autopsy case of sporadic amyotrophic lateral sclerosis, there were intracytoplasmic eosinophilic inclusions of the Bunina type in motor neurons. Electron microscopically, these bodies were observed as amorphous substances, irregularly deposited around the endoplasmic reticulum to form masses. Similar small masses were also visible in mitochondria. These substances did not stain for acid phosphatase. By analytical electron microscopy they contained silicon. Bunina bodies, therefore, seem to be due to deposition of some metabolite, but their nature is still obscure.
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PMID:Intracytoplasmic inclusions (Bunina bodies) in amyotrophic lateral sclerosis. 625 94

Twenty-two of 32 sporadic cases of amyotrophic lateral sclerosis had argyrophilic spheroids, 20 micrometers or larger, in the anterior horns of the spinal cords. The fine structure of these spherical bodies was characterized by interwoven, small bundles of 10 nm neurofilaments. Scattered mitochondria, vesicles and fragments of smooth endoplasmic reticulum were commonly found among the bundles of neurofilaments. The spheroids were present not only in the myelinated axons, but also in the perikarya of the anterior horn cells. In anterior horn neurons occasional fragments of rough endoplasmic reticulum, lipofuscin and even nuclei were found among the neurofilaments, in addition to the other components. Rarely, some filamentous accumulations contained unusual features such as paracrystalline arrays, polyglucosan bodies and honeycomb-like structures. Linear densities, associated with ribosome-like particles, were found scattered within focal collections of randomly arranged neurofilaments in some perikarya of two cases. Occasional mitochondria with regularly arranged short protrusions on the outer membrane were observed in the myelinated axons in one case.
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PMID:Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis. 654 Jul 99

Electron microscopic examination of the frontal cortex of a patient with amyotrophic lateral sclerosis (ALS) with 14-year prolongation of life by artificial ventilation of the lungs revealed heretofore undescribed cytoplasmic virus-like inclusions in neurons and glial cells as well as changes in the granular endoplasmic reticulum in astrocytes consisting of large accumulations of ribosomes in which regular arrays were frequently observed. The discovered inclusions were not similar to any identified viral inclusions but were similar to virus-like particles found in the muscle in ALS and viral inclusions in experimental poliomyelitis. The role of the observed inclusions and their etiological importance remain obscure.
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PMID:[Virus-like inclusions in the cells of the central nervous system in amyotrophic lateral sclerosis]. 718 5


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