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  • 1
    Electronic Resource
    Electronic Resource
    Pancreatic beta-cell secretory defect associated with mitochondrial point mutation of the tRNALEU(UUR) gene: a study in seven families with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) (1994)
    Springer
    Diabetologia 37 (1994), S. 818-825 
    Details
    ISSN: 1432-0428
    Keywords: C-peptide ; diabetes mellitus ; insulin secretion ; MELAS ; mitochondrial gene mutation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Recent evidence suggests possible linkage between diabetes mellitus and mitochondrial gene mutation. We surveyed mitochondrial tRNALEU(UUR) (3243) mutation in 7 mitochondrial encephalomyopathy, lactic acidosis and stroke-like episode (MELAS) families and identified 24 mutated subjects (7 MELAS probands and 17 non-MELAS relatives) as well as 11 non-mutant family members. An OGTT in the 24 mutant relatives revealed 14 diabetic subjects, 3 with impaired glucose tolerance and 7 with normal glucose tolerance and all non-mutant family members as having normal glucose tolerance. Insulinogenic index was significantly reduced in the mutant diabetic subjects and those with impaired and normal glucose tolerance in comparison with the normal control subjects and the non-mutant members. Urinary 24-h C-peptide immunoreactivity excretion was markedly reduced in the mutant diabetic subjects and those with normal and impaired glucose tolerance, compared with the control subjects and the non-mutant family members. Plasma C-peptide immunoreactivity 6 min after glucagon injection was markedly reduced in the mutant diabetic subjects and those with normal and impaired glucose tolerance compared with the control subjects and the non-mutant family members. Si, an index of insulin sensitivity of the four mutant subjects was within normal range. Islet cell antibodies were negative in sera of eight mutated diabetic subjects, 2 and 6 with impaired and normal glucose tolerance, respectively. Diabetic retinopathy and nephropathy were demonstrated in 7 (50%) and 12 (85.7%) of 14 mutant diabetic subjects, respectively. Neurosensory deafness was demonstrated in 12 (85.7%) of 14 mutated diabetic subjects, (66.7%) of 3 mutated impaired glucose tolerant subjects, but not detected in 6 mutated normal glucose tolerant subjects and 11 non-mutant family members. These findings suggest that the tRNALEU(UUR) mutation is associated with pancreatic beta-cell secretory defect of insulin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00404339
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  • 2
    Electronic Resource
    Electronic Resource
    Pancreatic beta-cell secretory defect associated with mitochondrial point mutation of the tRNA L E U ( U U R ) gene: a study in seven families with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) (1994)
    Springer
    Diabetologia 37 (1994), S. 818-825 
    Details
    ISSN: 1432-0428
    Keywords: Key words C-peptide, diabetes mellitus, insulin secretion, MELAS, mitochondrial gene mutation.
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Recent evidence suggests possible linkage between diabetes mellitus and mitochondrial gene mutation. We surveyed mitochondrial tRNALEU(UUR) (3243) mutation in 7 mitochondrial encephalomyopathy, lactic acidosis and stroke-like episode (MELAS) families and identified 24 mutated subjects (7 MELAS probands and 17 non-MELAS relatives) as well as 11 non-mutant family members. An OGTT in the 24 mutant relatives revealed 14 diabetic subjects, 3 with impaired glucose tolerance and 7 with normal glucose tolerance and all non-mutant family members as having normal glucose tolerance. Insulinogenic index was significantly reduced in the mutant diabetic subjects and those with impaired and normal glucose tolerance in comparison with the normal control subjects and the non-mutant members. Urinary 24-h C-peptide immunoreactivity excretion was markedly reduced in the mutant diabetic subjects and those with normal and impaired glucose tolerance, compared with the control subjects and the non-mutant family members. Plasma C-peptide immunoreactivity 6 min after glucagon injection was markedly reduced in the mutant diabetic subjects and those with normal and impaired glucose tolerance compared with the control subjects and the non-mutant family members. Si, an index of insulin sensitivity of the four mutant subjects was within normal range. Islet cell antibodies were negative in sera of eight mutated diabetic subjects, 2 and 6 with impaired and normal glucose tolerance, respectively. Diabetic retinopathy and nephropathy were demonstrated in 7 (50 %) and 12 (85.7 %) of 14 mutant diabetic subjects, respectively. Neurosensory deafness was demonstrated in 12 (85.7 %) of 14 mutated diabetic subjects, (66.7 %) of 3 mutated impaired glucose tolerant subjects, but not detected in 6 mutated normal glucose tolerant subjects and 11 non-mutant family members. These findings suggest that the tRNALEU(UUR) mutation is associated with pancreatic beta-cell secretory defect of insulin. [Diabetologia (1994) 37: 818–825]
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00404339
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  • 3
    Electronic Resource
    Electronic Resource
    Distinctive, rapid, and easy labeling of diffuse plaques in the Alzheimer brains by a new methenamine silver stain (1990)
    Springer
    Acta neuropathologica 79 (1990), S. 569-572 
    Details
    ISSN: 1432-0533
    Keywords: Senile plaques ; Methenamine silver stain ; Alzheimer-type dementia ; Down's syndrome ; Amyloid β protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We have developed a new methenamine silver (MS) stain for detecting diffuse plaques distinctively on paraffin-embedded tissue sections of Alzheimer-type dementia, Down'n syndrome, and mentally normal aged brains. This rapid and easy method selectively labels amyloid-related component of senile plaques, but not of kuru plaques found in Gerstmann-Sträussler syndrome. Our MS stain shows almost the same staining pattern as that of the β protein immunostaining with formic acid pretreatment. Therefore, new MS stain is appropriate to routine or screening studies for senile plaques including diffuse plaques.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296119
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  • 4
    Electronic Resource
    Electronic Resource
    Reexamination of granulovacuolar degeneration (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 340-345 
    Details
    ISSN: 1432-0533
    Keywords: Ubiquitin ; granulovacuolar degeneration ; ageing
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Granulovacuolar degeneration (GVD) in the hippocampal pyramidal neurons of Alzheimer-type dementia was examined. Immunohistochemical examinations showed that the majority of centrally located granules were positive for ubiquitin. Based on electron microscopic observations, morphogenesis of GVD is considered to be as follows. Slight-to-moderate amounts of electron-dense material appear in the cytoplasm at the early stage, and are then surrounded and demarcated by a two-layered membrane (probably from smooth endoplasmic reticulum). Following this some inner material is digested forming floccular and liquid-like materials, while undigested material remains as coarse electron-dense granules. Specifically, granulovacuoles are considered to be an age-related special type of autophagosome. Analytical electron microscopy disclosed that the granules in GVD contained some aluminum.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296544
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  • 5
    Electronic Resource
    Electronic Resource
    Widely distributed Bunina bodies and spheroids in a case of atypical sporadic amyotrophic lateral sclerosis (1991)
    Springer
    Acta neuropathologica 81 (1991), S. 349-353 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Bunina body ; Clarke's nucleus ; Onuf's nucleus ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report the autopsy findings of an 81-year-old patient with short-course sporadic amyotrophic lateral sclerosis lasting approximately 5 months. Pathological findings were probably very early. Light microscopy showed abundant eosinophilic Bunina type inclusions widely distributed not only in the motor neurons of the spinal cord and brain stem but also in neurons of the Onuf's and Clarke's nuclei. Fine structural study revealed that the inclusions seen in the Clarke's nuclei were identical to Bunina bodies observed in anterior horn cells. A direct connection between axonal swelling and perikaryon was often seen in the facial and hypoglossal nuclei and in the spinal cord. Ubiquitin-positive Lewy body-like inclusions and central chromatolysis-like changes were also found in the anterior horn cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00305880
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  • 6
    Electronic Resource
    Electronic Resource
    Ultrastructural localization of argyrophilic substances in diffuse plaques of Alzheimer-type dementia demonstrated by methenamine silver staining (1991)
    Springer
    Acta neuropathologica 81 (1991), S. 540-545 
    Details
    ISSN: 1432-0533
    Keywords: Senile dementia of the Alzheimer type ; Diffuse plaques ; Methenamine silver (MS) stain ; Electron micrograph
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The ultrastructure of argyrophilic substances in diffuse plaques of an Alzheimer-type dementia brain was examined using methenamine silver (MS) electron microscopy with the pre-embedding method. Electrondense substances, which were sparse aggregations of bundle-like structures with silver granules, were disseminated in the diffuse plaques. Comparison of serial MS and routine ultrathin sections revealed that diffuse plaques usually show scattered bundles of amyloid fibrils or amorphous materials, or both, between indistinct cell process membranes. Some of these processes were identified as astrocytic or dendritic in origin. A few degenerative neurites were frequently noted in large silver-positive areas but never in small areas. These findings suggest that the appearance of amyloid fibrils and amorphous materials between certain cell processes is a very morphological change in the process of senile plaque formation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310135
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  • 7
    Electronic Resource
    Electronic Resource
    Ultrastructure of the neuropil threads in the Alzheimer brain: their dendritic origin and accumulation in the senile plaques (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 368-374 
    Details
    ISSN: 1432-0533
    Keywords: Neuropil threads ; Alzheimer-type dementia ; tau protein ; Palred helical filaments ; Senile plaques
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Thread-like structures immunoreactive with paired helical filaments and tau antisera were demonstrated as mesh-works in the neocortices of five brains with Alzheimer-type dementia, but not in those of five normal aged control brains. The ultrastructure of the threads was examined using paired routine electron microscopic ultrathin sections and adjacent 0.4-μm-thick semithin sections, immunostained for β protein. Outside the β protein-positive senile plaques, neuropil threads appeared sporadically as small slender neurites, containing either regularly constricted or straight filaments. These neurites often showed dendritic profiles. Similar threads were also seen within the senile plaques. The threads were accumulated in amyloid fibril-rich primitive plaques, but not in amyloid fibril-poor diffuse plaques. The presence of these threads was closely associated with neurofibrillary tangle formation. Our findings suggest that wide-spread change of the neuropil neurites, neuropil threads or curly fibers, both outside and inside of the senile plaques are dendritic in origin and play an important role in the clinical manifestation of dementia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00307689
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  • 8
    Electronic Resource
    Electronic Resource
    Axonal swellings in the corticospinal tracts in amyotrophic lateral sclerosis (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 222-226 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Spheroid ; Corticospinal tract
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In 2 of 16 cases with sporadic amyotrophic lateral sclerosis (ALS) large numbers of axonal swellings were observed in the corticospinal tracts over a region extending from the posterior limbs of internal capsules to the bulbar pyramids. On electron microscopy, these axonal swellings were seen to consist of accumulations of neurofilaments and altered neuronal organelles (mitochondria and secondary lysosomes). Their morphology differed from the spheroids seen in the anterior horn in ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00308929
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  • 9
    Electronic Resource
    Electronic Resource
    Light and electron microscopic and immunohistochemical observations of the Onuf's nucleus of amyotrophic lateral sclerosis (1991)
    Springer
    Acta neuropathologica 81 (1991), S. 610-614 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Bunina body ; Onuf's nucleus ; Ubiquitin ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We examined the Onufrowicz nucleus (Onuf's nucleus) of ten sporadic amyotrophic lateral sclerosis (ALS) patients with light and electron microscopic and immunohistochemical methods. Neurons in the Onuf's nucleus of ALS patients were better preserved than those in anterior horn cells. However, some showed morphological changes in the nucleus, namely, central chromatolytic changes, Bunina bodies, ubiquitin-positive filaments and spheroids. The Onuf's neurons of ALS patients showed more argentophilia than those of non-ALS patients. Electron microscopic observations revealed that neurofilaments were relatively more numerous in the Onuf's neurons of ALS patients. Bunina bodies and degenerated neurites were also seen in the Onuf's nucleus. In conclusion, the Onuf's nucleus in sporadic ALS patients showed some morphological changes similar to those noted in anterior horn cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296370
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  • 10
    Electronic Resource
    Electronic Resource
    Extracellular neurofibrillary tangles associated with degenerating neurites and neuropil threads in Alzheimer-type dementia (1991)
    Springer
    Acta neuropathologica 81 (1991), S. 603-609 
    Details
    ISSN: 1432-0533
    Keywords: Alzheimer-type dementia ; Neurofibrillary tangles ; Neuropil threads ; Amyloid β/A4 protein ; Astroglia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We examined the cellular components of extracellular neurofibrillary tangles (E-NFT) in the hippocampal areas in cases with Alzheimer-type dementia. Immunohistochemically, the E-NFT were labeled for the C terminus of tau and glial fibrillary acidic protein. Moreover, the majority of the E-NFT was associated with intensely argyrophilic rods and with tau-and ubiquitin-immunoreactive dots. Ultrastructurally, the E-NFT consisted mainly of extracellular paired helical filaments (PHF) and astroglial processes. The extracellular PHF tended to be straighter and thinner. One third of the E-NFT was associated with small degenerating neurites containing many dense bodies and with neuropil threads containing PHF. These findings suggested that extracellular PHF promote both intense astroglial reaction and neuritic alteration, and that the E-NFT are continuously changing their morphology.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296369
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