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  • 1
    ISSN: 1573-6903
    Keywords: Proteolipid protein ; HAM/TSP ; T cell ; myelin ; HTLV-I
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To understand the immune mechanism suggested in HTLV-I-associated myelopathy (HAM/TSP), we investigated T cell response to proteolipid protein (PLP). Because of high autologous proliferative response (APR) of peripheral blood mononuclear cells (PBMC) in culture, the lymphocyte proliferation assay was not useful in this disease. Unexpectedly, however, APR was profoundly (70–98%) suppressed in 6 of 9 cases when PLP peptide 105-124 was added in the culture. PLP peptide 85-104 or 145-159 also suppressed APR in a few cases. Time course study showed that the peptide-mediated suppression became apparent after day 4 in culture. The results can be interpreted as that suppressor cells recognizing the PLP peptides were present in the PBMC of HAM/TSP patients and suppressed the APR as the consequence of antigen specific response. This may indicate that a T cell response to certain PLP determinants is involved in the pathomechanism of HAM/TSP at least in part. Molecular mimicry between PLP and HTLV-I mayaccount for the T cell sensitization to PLP in HAM/TSP.
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  • 2
    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.
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  • 3
    ISSN: 1572-994X
    Keywords: HTL V-I ; Mashhadi Jews ; saliva ; phylogenetic analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A new endemic focus of human T-lymphotropic virus type I (HTL V-I) was recently reported among Mashhadi Jews, a group of immigrants from northeastern Iran to Israel. We extracted DNAs from fresh peripheral blood mononuclear cells (PBMCs) and/or gargle mouthwash from 10 HTL V-I carriers, who consisted of members of one family, and HTL V-I-associated myelopathy (HAM) and adult T-cell leukemia (ATL) patients. Long terminal repeat (LTR) regions of proviral DNAs were sequenced and analyzed phylogenetically. In a phylogenetic tree, all the Mashhadi HTL V-I isolates belonged to subtype A, one of the three subtypes of the cosmopolitan type of HTL V-I, and made a tight cluster distinct from the other isolates of subtype A from Japan, India, the Caribbean Basin, and South America. Although a few nucleotide substitutions were observed among the clones sequenced, no characteristic sequence variation was found in different disease manifestations, even in one family or different sources of DNA preparation.
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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.
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  • 5
    ISSN: 1435-232X
    Keywords: Key words Adult onset ; Limb-girdle type ; Mitochondrial myopathy ; Familial ; Mitochondrial DNA ; np8291 A-to-G substitution
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract We analyzed mitochondrial DNA (mtDNA) from 7 patients in four families with adult onset limb-girdle type mitochondrial myopathy to clarify their genetic background. The patients, 2 men and 5 women, showed common clinical features, characterized by isolated skeletal myopathy, high serum creatine kinase level, ragged-red fibers and cytochrome c oxidase-defective fibers. Analysis of muscle biopsy specimens indicated that cytochrome c oxidase activity was decreased relative to that of citrate synthase in 5 of the 7 patients. Southern blotting and direct sequence analyses showed an A-to-G homoplasmic transition at np8291 and intergenic COII/tRNA(Lys) 9bp deletion in all patients. This substitution was detected in only 2 of 600 control individuals including healthy subjects and patients with other neuromuscular disorders; these 2 individuals had diabetes mellitus and myotonic dystrophy, respectively. Consequently, the mtDNA transition at np8291 was a rare polymorphism. However, the 7 patients we studied had identical clinical, pathological, biochemical, and genetic features. Therefore, limb-girdle type mitochondrial myopathy with this rare polymorphism may form a subgroup of adult onset mitochondrial myopathy.
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  • 6
    ISSN: 1435-232X
    Keywords: myotonic dystrophy ; unstable CTG repeat sequence ; anticipation ; dynamic mutation ; contraction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary To study the characteristics, if any, of unstable CTG repeat sequence in Japanese myotonic dystrophy (DM), we analyzed DNA from 351 at risk individuals (including affected and non-affected carriers and their descendants) from 105 families in Japan. A total of 93 DM families (196 affected and 116 unaffected individuals), including 84 DM parentchild pairs (44 father-child and 40 mother-child pairs), were examined, many of which had been previously tested by linkage analysis. We detected unstable CTG repeat mutations between 0.15 kb and 8.7 kb in size. The size of the mutation correlated with the age of onset of symptoms. There was a significant difference in DM allele size among the four groups (congenital, juvenile onset, classical, and minimal). Congenital DM had on average the largest repeat sizes. Comparison of parent-child pairs showed that most offspring had a larger repeat size than their parents, with only 2 of 84 showing a definite decrease in repeat size. The correlation coefficients for maternal and paternal transmission were 0.41 and 0.15, respectively. The parental age (maternal and paternal) did not correlate with intergenerational change of repeat. These observations are similar to those reported in Caucasians.
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  • 7
    ISSN: 1573-6865
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
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  • 8
    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.
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  • 9
    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.
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  • 10
    ISSN: 1432-0533
    Keywords: Key words Adhesion molecules ; HTLV-1-associated ; myopathy/tropical spastic paraparesis ; Monocyte ; chemoattractant protein-1 ; Vascular cell adhesion ; molecule-1 ; Very late antigen-4
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Leukocyte adhesion molecules to endothelium plays an important role in the pathogenesis of inflammatory diseases, including HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP). To help define the role of adhesion molecules in HAM/TSP, we studied the expression of lymphocyte function-associated antigen-1 (LFA-1), Mac-1, very late antigen-4 (VLA-4), Sialyl Lewisx (SLex), intercelluar adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), endothelial leukocyte adhesion molecule-1 (ELAM-1) and monocyte chemoattractant protein-1 (MCP-1) in the spinal cord lesions of HAM/TSP. The results indicate that spinal cord lesions of HAM/TSP have greater VCAM-1 expression on endothelium compared with those of controls. Infiltrating mononuclear cells, especially perivascular lesions, expressed VLA-4. Although the expression of ICAM-1 in the spinal cords was not distinctive between HAM/TSP and controls, infiltrating mononulcear cells in the spinal cords of HAM/TSP strongly expressed LFA-1 and Mac-1. ELAM-1 was expressed on endothelium in the inactive-chronic lesions from three of five HAM/TSP, but was not detectable in the spinal cords of controls. SLex reaction was detectable on occasional perivascular cells in the spinal cord of HAM/TSP, but not in those of controls. MCP-1 was detectable on perivascular infiltrating cells and vascular endothelium in active-chronic lesions. This study suggests that VLA-4/VCAM-1 interaction may play an important role for lymphocyte migration into the central nervous system (CNS), and MCP-1 may also be involved in inflammatory cell recruitment to the CNS in HAM/TSP.
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