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  • ligand  (1)
  • mRNA  (1)
  • muscle, in situ hybridization  (1)
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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 100 (2000), S. 521-527 
    ISSN: 1432-0533
    Keywords: Key words Polymyositis ; Lymphotoxin ; Perforin ; Fas ; ligand
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Polymyositis (PM) is a cell-mediated autoimmune disease. Perforin (PF), Fas ligand (FasL) and TNF-α are considered to be important factors in cytotoxic T lymphocyte-mediated cell injury, and several studies have established a role of lymphotoxin (LT) in T helper type 1 (Th1)-induced cell-mediated autoimmune diseases. In the present study, to determine how LT, PF and FasL are involved in the pathogenesis of PM, we used immunohistochemical staining (IHC), reverse transcription polymerase chain reaction (RT-PCR), and in situ hybridization (ISH) on muscle specimens from patients with PM, amyotrophic lateral sclerosis (ALS), myotonic dystrophy (MyD) and controls (NC). There were many mononuclear cells (MNCs) immunoreactive for LT and some for PF and FasL within the fasciculus in PM muscles. On the other hand, only few or no LT-, PF- and FasL-positive cells were detected in MyD, ALS and NC muscles. The results of mRNA expression of these three molecules with RT-PCR were consistent with those using IHC methods. The number of MNCs positive for LT with ISH was far higher in PM compared to MyD, ALS and NC (P 〈 0.05 or 0.01). The MNCs located in the connective tissue or in the vicinity of necrotizing or non-necrotizing muscles were mainly LT mRNA and CD4 positive, while MNCs invading the non-necrotic fibers were mainly LT mRNA and CD8 positive. Our results indicated that the expression of LT was up-regulated in PM, and LT plays an important role in muscle injury and orchestrating the inflammatory reaction in PM.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1573-6903
    Keywords: GDNF, GDNFR-α ; mRNA ; motor neuron disease ; muscle, in situ hybridization
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The mRNA expression levels of GDNF, GDNFR-α and RET were examined in the muscles of amyotrophic lateral screlosis (ALS) and X-linked spinal and bulbar muscular atrophy (SBMA). GDNF mRNA levels were significantly elevated to variable extent in the diseased muscles compared to control muscles, although they were not specific to the type of the diseases. The diseased muscles also have a different expression pattern of GDNF mRNA isoforms from controls. GDNF mRNA expression, however, tended to reduce in advanced muscle pathology. On the other hand, GDNFR-α mRNA levels were not changed significantly on expression levels in the diseased muscles. In situ hybridization study revealed that GDNF and GDNFR-α mRNAs were localized in subsarcolemmal space of muscle cells. RET mRNA was not detected in control nor diseased muscles. These results suggest that the elevated muscle GDNF acts as a trophic signal for motor neurons of motor neuron diseases, implying a possible therapeutic implication of GDNF to this type of diseases.
    Type of Medium: Electronic Resource
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