Subunit 1 of the prefoldin chaperone complex is required for lymphocyte development and function |
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Authors: | Cao Shang Carlesso Gianluca Osipovich Anna B Llanes Joan Lin Qing Hoek Kristen L Khan Wasif N Ruley H Earl |
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Institution: | Department of Microbiology and Immunology, Vanderbilt University, School of Medicine, Nashville, TN 37232, USA. |
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Abstract: | Prefoldin is a hexameric chaperone that facilitates posttranslational folding of actins and other cytoskeletal proteins by the Tcp1-containing ring complex chaperonin, TriC. The present study characterized mice with a null mutation in Pfdn1, which encodes the first subunit of the Prefoldin complex. Pfdn1-deficient mice displayed phenotypes characteristic of defects in cytoskeletal function, including manifestations of ciliary dyskinesia, neuronal loss, and defects in B and T cell development and function. B and T cell maturation was markedly impaired at relatively early stages, namely at the transitions from pre-pro-B to pre-B cells in the bone marrow and from CD4-CD8- double-negative to CD4+CD8+ double-positive T cells in the thymus. In addition, mature B and T lymphocytes displayed cell activation defects upon Ag receptor cross-linking accompanied by impaired Ag receptor capping in B cells. These phenotypes illustrate the importance of cytoskeletal function in immune cell development and activation. |
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