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  • 1
    Electronic Resource
    Electronic Resource
    Hydrocyanation. IV. New hydrocyanation methods using hydrogen cyanide and an alkylaluminum, and an alkylaluminum cyanide (1972)
    s.l. : American Chemical Society
    Journal of the American Chemical Society 94 (1972), S. 4635-4643 
    Details
    ISSN: 1520-5126
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1021/ja00768a037
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  • 2
    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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  • 3
    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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  • 4
    Electronic Resource
    Electronic Resource
    NACP/α-synuclein and tau constitute two distinctive subsets of filaments in the same neuronal inclusions in brains from a family of parkinsonism and dementia with Lewy bodies: double-immunolabeling fluorescence and electron microscopic studies (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 115-121 
    Details
    ISSN: 1432-0533
    Keywords: Key wordsα-Synuclein ; Filament pathology ; Lewy body ; NACP ; Tau
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The co-localization of NACP/α-synuclein and tau epitopes was examined in the brain stem and hippocampal formation in two patients from a family of autosomal dominant parkinsonism and dementia with Lewy bodies (LBs) without two reported missense mutations in the NACP gene. Double-labeling immunofluorescence study revealed that some brain stem LBs, cortical LBs, pale bodies, Lewy-related neurites, and neurofibrillary tangles expressed both NACP epitopes and the PHF tau AT8 epitope. Double-immunolabeling electron microscopy demonstrated that the NACP antibody selectively labeled 9- to 13-nm-thick straight filaments (LB filaments), whereas AT8 recognized twisted tubules with 80- to 100-nm-interval constrictions in the same neuronal inclusions. We show that NACP and tau aggregate into different filamentous components even if both proteins are incorporated into the same inclusions.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050002
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  • 5
    Electronic Resource
    Electronic Resource
    Alzheimer's neurofibrillary tangles are penetrated by astroglial processes and appear eosinophilic in their final stages (1987)
    Springer
    Acta neuropathologica 72 (1987), S. 214-217 
    Details
    ISSN: 1432-0533
    Keywords: Neurofibrillary tangles ; Senile dementia of Alzheimer type ; Glial fibrillary acidic protein ; Astrocytes ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Alzheimer's neurofibrillary tangles (ANT) in the hippocampal area were studied immunohistochemically using antisera against glial fibrillary acidic protein (GFAP) and S-100 protein in 48 patients with or without dementia between 52 and 92 years old. In 27 of the 38 brains that developed ANT in the hippocampal area, some ANT were immunostained with these antisera. Flame-shaped or globose-shaped immunostains were occasionally continuous with astroglial cell bodies and processes. They appeared particularly in the entorhinal cortex, subiculum and CAl. The ANT, immunostained with GFAP and S-100 antisera, apparently correspond to slightly eosinophilic tangles in H&E sections and to less argentophilic tangles in silver-impregnated sections in all of the 27 brains. ANT of another 11 brains were consistently negative with these antisera. The GFAP-positive eosinophilic tangles were encountered in the brains of older patients (P〈0.01) and with more abundant formation of ANT (P〈0.001). This alteration was present in all of the 20 brains with more than 100 ANT per section and none of the eight brains with less than 10 ANT. These findings suggest that in the last stages, ANT are penetrated by eosinophilic processes of astrocytes, and appear eosinophilic, and that the presence of GFAP-positive eosinophilic tangles indicates the abundant formation of ANT.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00691092
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  • 6
    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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  • 7
    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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  • 8
    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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  • 9
    Electronic Resource
    Electronic Resource
    Diffuse type of senile plaques in the cerebellum of Alzheimer-type dementia demonstrated byβ protein immunostain (1989)
    Springer
    Acta neuropathologica 77 (1989), S. 314-319 
    Details
    ISSN: 1432-0533
    Keywords: Alzheimer-type dementia ; Senile plaques ; β Protein ; Formic acid treatment ; Cerebellum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We studied senile plaques (SP) in the cerebella of six autopsied subjects with Alzheimer-type dementia (ATD) and ten non-ATD autopsied subjects between the ages of 78 and 90. Neither SP nor amyloid angiopathy (AA) was observed in any of the non-ATD subjects. In the four of the six ATD subjects, diffuse plaques in the molecular layer were seen as ill-defined areas of fine fibrillar materials by β protein immunostaining with formic acid pretreatment, the modified Bielschowsky stain, and periodic acid-methenamine silver (PAM) stain. The plaques were not visible with Bodian, Congo red, or periodic acid-Schiff stains. Compact plaques in the Purkinje cell or in the granular cell layers were found in three of the six subjects. Their amyloid core was often surrounded by areolar amyloid deposits. AA was observed in three of the six subjects. The argyrophilia of the diffuse and compact plaques, demonstrated by the modified Bielschowsky and PAM stains, became undetectable when the sections were first treated with formic acid. Such treatment made the plaques immunoreactive with β protein antiserum. The findings suggested that cerebellar diffuse plaques and compact plaques consist mainly of an amyloid component, and are characteristic of ATD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687584
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  • 10
    Electronic Resource
    Electronic Resource
    A variety of cerebral amyloid deposits in the brains of the Alzheimer-type dementia demonstrated byβ protein immunostaining (1988)
    Springer
    Acta neuropathologica 76 (1988), S. 541-549 
    Details
    ISSN: 1432-0533
    Keywords: β Protein ; Senile plaques ; Amyloid ; Alzheimer ; Dementia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We studied cerebral amyloid deposits in the hippocampal area immunohistochemically, using antiserum to syntheticβ peptide (1–28) in 66 patients with or without dementia and aged 17 to 91 years old. Senile plaques (SP) and amyloid angiopathy (AA) were detected in 36 (55%) and 19 (29%) patients, respectively. Also, cerebral amyloid deposits from the brains of seven patients with dementia and five patients without were studied in serial sections stained with Bodian, modified Bielschowsky, Congo red, andβ protein immunostain. In the patients with Alzheimer-type dementia (ATD) diffuse plaques, typical of this group, were stained withβ protein antiserum but not with Bodian stain, because the plaques were devoid of abnormally swollen neuritic processes. The diffuse plaques often contained one or more neuronal cell bodies. As well as primitive and classic plaques and AA, theβ protein immunostain demonstrated small deposits among the SP, small stellate deposits of layer 1, subpial fibrillar deposits, and focal cribriform deposits of parasubiculum, which may be new types of amyloid deposits. Amyloid plaques within the subcortical white matter were only found in ATD brains. In the non-demented patients various kinds of SP, including diffuse and compact ones, were immunostained. They tended to be small and few.β protein immunostain with formic acid pretreatment is a useful method for the identification of a variety of senile cerebral amyloid deposits.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00689591
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