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  • 1
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of bone and mineral metabolism 7 (1989), S. 42-48 
    ISSN: 1435-5604
    Keywords: Male hypogonadal osteoporosis ; Slipped capital femoral epiphysis ; Testosterone ; GH ; Somatomedin C
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two males with bone abnormalities associated with hypogonadotropic hypogonadism are reported. Case 1, 28 years old male, developed growth disturbance at the age of eight years, after suffering from tuberculous meningitis. No secondary sex characteristics appeared and fractures occurred at five times. Case 2, 29 years old male, also suffered from growth disturbance from around the age of 6 years, without appearance of secondary sex characteristics even after puberty. Bone X-ray studies and bone biopsy revealed marked osteoporosis in Case 1, while in Case 2, slipped capital femoral epiphysis was also noted with mild osteoporosis. In these two cases, osteoporosis is associated with eunuchoidism, in agreement of the concept of so-called “male hypogonadal osteoporosis”. Both patients showed insufficient secretion of somatomedin C, testosterone and growth hormone (GH) with insulin tolerance test and arginine tolerance test. The insufficient secretion of LH and FSH with LH-RH tolerance test was also revealed in both cases. The decrease of GH and somatomedin C was quite pronounced in Case 1, whereas the fall of testosterone was more conspicuous in Case 2. The imbalance between these hormone deficiencies might lead to different expression of bone abnormalities.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0878
    Keywords: Cholinesterase ; Muscle, striated ; Regeneration ; Myopathy ; Neuromuscular junction ; Nuclear inclusion ; Rat (Sprague-Dawley)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary In the course of ultrastructural investigations of motor endplate pathology mediated by calcium ions, intranuclear sarcoplasmic inclusions, either membrane-free (true type) or membrane-delimited (false type), were observed during chronic daily high-dose exposure to the anticholinesterase neostigmine. At the stage in which subjunctional components, including soleplate nuclei, were severely damaged (day 7), the true nuclear inclusions were frequently associated with the disrupted nuclear envelope (fragmentation, vesiculation etc.) and nuclear pores. At a subsequent stage, in which muscle repair was accelerated and most soleplatenuclei were less severely affected (day 21), formation of the false inclusions in these nuclei was enhanced. Analysis of serial sections of the less severely affected nuclei, where only a true inclusion type was present, revealed no sign of invaginated nuclear envelopes or other membranes enclosing the inclusions. Our findings indicate that morphogenesis of true inclusions depends upon the severity of nuclear degeneration, i.e., in severely affected nuclei there is disruption in the nuclear envelope and/or nuclear pores, while in less severely affected nuclei, either a pinched-off invagination or diffusion of excessive sarcoplasmic proteins into the nucleus via nuclear pores occurs.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1435-232X
    Keywords: myotonic dystrophy ; DNA diagnosis ; apolipoprotein CII (APOC2) ; D19S19 ; restriction fragment length polymorphisms (RFLPs)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Myotonic dystrophy (DM) is a genetic disease inherited by an autosomal dominant trait and characterized by multi-organ disorders. Although its biochemical basis has been unknown, the DM locus is closely linked to D19S19 and APOC2 on the long arm of chromosome 19 both in Japanese and Caucasian populations. Linkage studies of Japanese DM families using these polymorphic DNA markers detected two asymptomatic gene carriers in two unrelated families.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 0003-276X
    Keywords: Life and Medical Sciences ; Cell & Developmental Biology
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Medicine
    Notes: To investigate annulate lamellae (AL) with nuclear changes, ultrastructural time course studies were done of the reversible end-plate myopathy in the soleus muscles of rats exposed chronically to a high dose of the anticholinesterase drug neostigmine. At the earliest stage (2 hours) in which severe subjunctional damage involving a nuclear lesion (nuclear pyknosis) was prominent, AL profiles were undetectable. At the intermediate stage (7, 21 days), in which the subjunctional organelles tended to cluster and nuclear pyknosis often accompanied degenerative features, most AL laid near the surface of the abnormal nuclei, where there were signs of elimination or formation of pores, as well as a progression of changes leading to the loss of pores. At the late stage (56 days), in which muscle repair was nearly completed and euchromatic nuclei usually were found, there was a concomitant enhanced formation of false nuclear inclusions and of the AL profiles in these sites.Pores are packed on nuclear envelopes, which detach themselves from the nucleus and take the same profile as AL. This phenomenon can be envisioned not only in the euchromatic but also in the abnormal (pyknotic or degenerated) nuclei. Following nuclear reconstitution, the nuclear envelope folding is accelerated to induce the usual pattern of AL organization, i.e., budding from the invaginated nuclear envelope.
    Additional Material: 10 Ill.
    Type of Medium: Electronic Resource
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