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Molecular and clinical diversity in paraneoplastic immunity to Ma proteins.
Ann Neurol. 2001 Sep; 50(3):339-48.AN

Abstract

Antibodies to Ma1 and Ma2 proteins identify a paraneoplastic disorder that affects the limbic system, brain stem, and cerebellum. Preliminary studies suggested the existence of other Ma proteins and different patterns of immune response associated with distinct neurologic symptoms and cancers. In this study, our aim was to isolate the full-length sequence of Ma2 and new family members, identify the major autoantigen of the disorder, and extend the dinical-immunological analysis to 29 patients. Sera from selected patients were used to probe a brainstem cDNA library and isolate the entire Ma2 gene and a new family member, Ma3. Ma3 mRNA is ubiquitously expressed in brain, testis, and several systemic tissues. The variable cellular expression of Ma proteins and analysis of protein motifs suggest that these proteins play roles in the biogenesis of mRNA. Immunoblot studies identify Ma2 as the major autoantigen with unique epitopes recognized by all patients' sera. Eighteen patients had antibodies limited to Ma2: they developed limbic, hypothalamic, and brainstem encephalitis, and 78% had germ-cell tumors of the testis. Eleven patients had antibodies to Ma2 and additional antibodies to Ma1 and/or Ma3; they usually developed additional cerebellar symptoms and more intense brainstem dysfunction, and 82% of these patients had tumors other than germ-cell neoplasms. Overall, 17 of 24 patients (71%) with brain magnetic resonance imaging studies had abnormalities within or outside the temporal lobes, some as contrast-enhancing nodular lesions. A remarkable finding of immunity to Ma proteins is that neurologic symptoms may improve or resolve. This improvement segregated to a group of patients with antibodies limited to Ma2.

Authors+Show Affiliations

Department of Neurology, University of Arkansas for Medical Sciences, Little Rock 72205, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

11558790

Citation

Rosenfeld, M R., et al. "Molecular and Clinical Diversity in Paraneoplastic Immunity to Ma Proteins." Annals of Neurology, vol. 50, no. 3, 2001, pp. 339-48.
Rosenfeld MR, Eichen JG, Wade DF, et al. Molecular and clinical diversity in paraneoplastic immunity to Ma proteins. Ann Neurol. 2001;50(3):339-48.
Rosenfeld, M. R., Eichen, J. G., Wade, D. F., Posner, J. B., & Dalmau, J. (2001). Molecular and clinical diversity in paraneoplastic immunity to Ma proteins. Annals of Neurology, 50(3), 339-48.
Rosenfeld MR, et al. Molecular and Clinical Diversity in Paraneoplastic Immunity to Ma Proteins. Ann Neurol. 2001;50(3):339-48. PubMed PMID: 11558790.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Molecular and clinical diversity in paraneoplastic immunity to Ma proteins. AU - Rosenfeld,M R, AU - Eichen,J G, AU - Wade,D F, AU - Posner,J B, AU - Dalmau,J, PY - 2001/9/18/pubmed PY - 2001/10/5/medline PY - 2001/9/18/entrez SP - 339 EP - 48 JF - Annals of neurology JO - Ann Neurol VL - 50 IS - 3 N2 - Antibodies to Ma1 and Ma2 proteins identify a paraneoplastic disorder that affects the limbic system, brain stem, and cerebellum. Preliminary studies suggested the existence of other Ma proteins and different patterns of immune response associated with distinct neurologic symptoms and cancers. In this study, our aim was to isolate the full-length sequence of Ma2 and new family members, identify the major autoantigen of the disorder, and extend the dinical-immunological analysis to 29 patients. Sera from selected patients were used to probe a brainstem cDNA library and isolate the entire Ma2 gene and a new family member, Ma3. Ma3 mRNA is ubiquitously expressed in brain, testis, and several systemic tissues. The variable cellular expression of Ma proteins and analysis of protein motifs suggest that these proteins play roles in the biogenesis of mRNA. Immunoblot studies identify Ma2 as the major autoantigen with unique epitopes recognized by all patients' sera. Eighteen patients had antibodies limited to Ma2: they developed limbic, hypothalamic, and brainstem encephalitis, and 78% had germ-cell tumors of the testis. Eleven patients had antibodies to Ma2 and additional antibodies to Ma1 and/or Ma3; they usually developed additional cerebellar symptoms and more intense brainstem dysfunction, and 82% of these patients had tumors other than germ-cell neoplasms. Overall, 17 of 24 patients (71%) with brain magnetic resonance imaging studies had abnormalities within or outside the temporal lobes, some as contrast-enhancing nodular lesions. A remarkable finding of immunity to Ma proteins is that neurologic symptoms may improve or resolve. This improvement segregated to a group of patients with antibodies limited to Ma2. SN - 0364-5134 UR - https://www.unboundmedicine.com/medline/citation/11558790/Molecular_and_clinical_diversity_in_paraneoplastic_immunity_to_Ma_proteins_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0364-5134&date=2001&volume=50&issue=3&spage=339 DB - PRIME DP - Unbound Medicine ER -