Cytotoxic T lymphocyte-mediated cell death in paraneoplastic sensory neuronopathy with anti-Hu antibody.
Paraneoplastic sensory neuronopathy (PSN) has been shown to harbor characteristic anti-neuronal autoantibody 'anti-Hu' in their sera and cerebrospinal fluid. Creation of animal models exhibiting clinical or pathological features seen in PSN by means of passive transfer of anti-Hu positive IgG has not been achieved. Although, anti-Hu antibody was shown to induce neuronal cell lysis in vitro, this result has not been reproduced so far. Since prominent T cell infiltration are seen in the central nervous system and posterior spinal ganglion of the patients with anti-Hu syndrome, we studied cytotoxic T cell (CTL) activity in peripheral mononuclear cells from a patient with PSN harboring anti-Hu antibody. The activated CD8+ T cells from the patient's venous blood were shown to lyse her own fibroblasts which were incubated with interferon-gamma to induce HLA class I molecules on their surface and the recombinant HuD protein was injected into the cells by microinjector. This is the first report showing the existence of CTL in a patient with PSN.
Tanaka
K
K
Department of Neurology, Brain Research Institute, Niigata University, Japan. keiko@bri.niigata-u.ac.jp
Tanaka
M
M
Inuzuka
T
T
Nakano
R
R
Tsuji
S
S
D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid*; D003602:Cytotoxicity, Immunologic; D051959:ELAV Proteins; D000067779:ELAV-Like Protein 4; D006801:Humans; D009419:Nerve Tissue Proteins; D010257:Paraneoplastic Syndromes / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000503:physiopathology; D016601:RNA-Binding Proteins / Q000276:immunology*; D011994:Recombinant Proteins / Q000276:immunology; D012678:Sensation Disorders / Q000209:etiology / Q000276:immunology*; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology*
D001323:Autoantibodies; D051959:ELAV Proteins; D000067779:ELAV-Like Protein 4; C498010:ELAVL4 protein, human; D009419:Nerve Tissue Proteins; D016601:RNA-Binding Proteins; D011994:Recombinant Proteins