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Assessment of free light chains in HCV-positive patients with mixed cryoglobulinaemia vasculitis undergoing rituximab treatment. Muscle-specific kinase myasthenia gravis IgG4 autoantibodies cause severe neuromuscular junction dysfunction in mice. Umberto Basile and Mariapaola Marino equally contributed to this work.

Paola Chiara Bartoccioni

Our goal, however, was to conduct a pilot study on serum levels of Barhoccioni and IgG subclasses in an organ-specific autoimmune disease like MG. Being a by-product of intact immunoglobulin synthesis, they may represent a marker of overall B cell activity, particularly in those diseases where there is an increased formation of immune complexes like systemic autoimmune disorders.

National Center for Biotechnology InformationU. Longitudinal studies, evaluating changes in these cell subsets in different Bartoccioji phases and in response to treatment, will clarify their role as markers of disease activity [ 21 ]. Am J Physiol-renal, 12F Serum free light chains as biomarkers for systemic lupus erythematosus disease activity. J Physiol-London, Pt 24 Clinical and Experimental Immunology. Along with age at onset and thymus pathology, the auto- abs status is used in the definition of disease subgroups [ 17 ].


B cells and plasmablasts seem to be key players in several autoimmune, RTX-responsive disorders [ 32 ].

Structural bases for the interaction and stabilization of the human amino acid transporter LAT2 with its ancillary protein 4F2hc. Diabetologia, 52 8 J Biol Chem, 18 vartoccioni, Guidelines for treatment of autoimmune neuromuscular transmission disorders. Epidemiological and immunological profile of muscle-specific kinase myasthenia gravis in Greece.

Substrate binding tunes conformational flexibility barttoccioni kinetic stability of an amino acid antiporter. Role of transmembrane domain 8 in substrate selectivity and translocation of SteT, a member of the L-amino acid transporter LAT family.

Journal List J Immunol Res v.

Amino Acid Transporters and Disease. Serological levels of free immunoglobulin light chains FLCsproduced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab RTX therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus.

It is yet to be clarified whether the early use of rituximab could prevent the permanent bulbar bartoccilni, which constitutes a relevant disability in these patients. Several reports, analysing T and B cell subsets in MG patients, showed a disequilibrium between follicular and regulatory T cells together with a lower frequency of regulatory B cells.


We are conscious that there are limitations to our study. Table 2 IgG subclasses distribution in patients and controls. Open in a separate window. Auto-abs bind to well-defined antigens in the postsynaptic membrane at the neuromuscular junction and impair nerve-muscle transmission, which in turn induces muscle weakness and excessive fatigability.

Bartoccioni pdf

We were able to measure retrospectively specific auto-abs and FLCs in two blood samples of 4 MuSK-MG patients treated with rituximab 10—13, Supplementary Table 2collected at different time points before first infusion 8—60 months and 3—8 weeks after first RTX treatment Table 3. The human protein is part of a multimeric complex regulating Calcium transport across the cell membrane, thus controlling B lymphocytes activation and proliferation; accordingly, RTX binding to CD20 interferes with these processes [ 28 ].

Circulating Ig-producing long-lived plasma cells are not depleted by RTX [ 29 ]. A truly bartocciomi biomarker should meet strict criteria: Roles of plasmablasts and B cells in IgG4-related disease: The small SLC43 family: