Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Expression of proteins associated with immune function was investigated immunohistochemically in postmortem brain and spinal cord of patients with amyotrophic lateral sclerosis (ALS). Reactive microglia/macrophages displaying high levels of leukocyte common antigen (LCA), the immunoglobulin receptor Fc gamma R1, lymphocyte function associated molecule-1 (LFA-1), the complement receptors CR3 and CR4, the class II major histocompatibility complex molecules HLA-DR, HLA-DP and HLA-DQ and common determinants of the class I HLA-A,B,C complex were abundant in affected areas in ALS. These areas included the primary motor cortex, motor nuclei of the brain stem, the anterior horn of the spinal cord, and the full extent of the corticospinal tract. A significant number of T lymphocytes of the helper/inducer (CD4+) and cytotoxic/suppressor (CD8+) subtypes were observed marginating along the walls of capillaries and venules and extending into the parenchyma of affected areas. Clusters of complement activated oligodendroglia as well as degenerating neurites positive for C3d and C4d were frequently detected in ALS-affected areas. These data provide evidence of immune-effector changes in ALS. They are consistent with an autoimmune or slow virus theory of the disorder, but may reflect only secondary changes.
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PMID:Immunologic reactions in amyotrophic lateral sclerosis brain and spinal cord tissue. 134 73

C3H/He skin allografts are significantly prolonged in ALS-treated B6AF1 mice by the injection of 50 x 10(6) C3H/He spleen cells 1 week postgrafting. To identify and characterize the spleen cell active in promoting graft survival, C3H/He spleen cells were separated on a discontinuous Percoll gradient and the various fractions were assayed for the ability to prolong skin graft survival in ALS-treated B6AF1 mice. In addition, unfractionated spleen and spleen fractions were depleted of specific cell populations before injection to determine the effect of various cell populations on graft prolongation. The active cell was recovered primarily in the 52.5% Percoll fraction. Cells in the 60% fraction also had a graft-prolonging effect, but not as significant as that of the 52.5% fraction. Depletion of Thy 1, Ia and Ig-positive cells from unfractionated spleen or spleen fractions did not decrease the graft-prolonging effect. Both Fc gamma R-positive and Fc gamma R-negative cells prolonged graft survival, but the Fc gamma R- cells were the most effective. In contrast to the effect of spleen cells, lymph node cells and thymocytes are relatively ineffective in prolonging graft survival in ALS-treated mice. When lymph node lymphocytes and thymocytes were separated on a Percoll gradient, the cell population active in prolonging graft survival was recovered primarily in the 52.5% fraction. Treatment of the 52.5% fraction of lymph node lymphocytes or thymocytes with monoclonal antibody to Thy 1 before injection abrogated the graft-prolonging effect. These results indicate that the spleen cell(s) active in prolonging graft survival in ALS-treated mice is a non-T, non-B cell, as it lacks Thy 1, Ia, and Ig surface markers. Both Fc gamma R+ and Fc gamma R- spleen cells are effective in prolonging grafts, but Fc gamma R- cells are the most effective. In contrast, the active cell in lymph node and thymus is Thy 1-positive, indicating that it is a T lymphocyte.
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PMID:Characterization of spleen cells capable of inducing unresponsiveness in ALS-treated mice. 167 Sep 69

Elements consistent with a cell mediated immune response were identified immunohistochemically in amyotrophic lateral sclerosis (ALS) spinal cord and Alzheimer disease (AD) hippocampus. T helper/inducer and cytotoxic/suppressor lymphocytes were detected in affected tissues in both diseases. In addition, abundant reactive microglia were found expressing the major histocompatibility glycoproteins HLA-A,B,C and HLA-DR, as well as receptors for the Fc chain (Fc gamma R1), for complement 3 and 4, and for vitronectin. In AD, the complement proteins C1q, C4d, C3d and C5b-9 were found on dystrophic neurites, neuropile threads and some neurofibrillary tangles. In ALS, the only complement proteins identified were C4d and C3d. The integrin ligands vitronectin and ICAM-1 were also identified in affected tissues in both diseases.
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PMID:Reactions of the immune system in chronic degenerative neurological diseases. 193 84

By using quantitative multiparameter microscopic imaging we demonstrate concentration of two peripheral mononuclear blood leukocyte types expressing the Fc gamma RIII receptor for immunoglobulin G in a clinical subgroup of amyotrophic lateral sclerosis (ALS, n = 9) showing bulbar palsy (ALSBP) and/or predominant involvement of the upper motor neuron (ALSC). Triple fluorescence staining and overlay with phase contrast images (4 parameters) reveals that cell type 1 co-expresses Fc gamma RIII (CD16), CD8 and CD57 surface antigens (ALSC 50 +/- 33.6 cells/microliters, P = 0.0012; ALSBP 16.5 +/- 32.4, P = 0.029). This cell type is not observed in healthy individuals (n = 8) and is only insignificantly increased (P > 0.05) in neurological disease controls (stroke, n = 3, 2.1 +/- 3.7; polymyositis, n = 6, 1.5 +/- 4.0 cells/microliters) and in ALS cases with peripheral symptoms (ALSP n = 12: 7.6 +/- 8.7). Cell type 2 co-expresses Fc gamma RIII (CD16) and CD8, but is negative for CD57 (ALSC 60.1 +/- 19.3; ALSBP 24.2 +/- 28.0 cells/microliters). These findings are consistent with previous reports on IgG isotype changes and immune-cell invasion of the motor system in ALS.
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PMID:Detection by 4-parameter microscopic imaging and increase of rare mononuclear blood leukocyte types expressing the Fc gamma RIII receptor (CD16) for immunoglobulin G in human sporadic amyotrophic lateral sclerosis (ALS). 857 89