| First Author | Cao S | Year | 2008 |
| Journal | J Immunol | Volume | 181 |
| Issue | 1 | Pages | 476-84 |
| PubMed ID | 18566413 | Mgi Jnum | J:137415 |
| Mgi Id | MGI:3799445 | Doi | 10.4049/jimmunol.181.1.476 |
| Citation | Cao S, et al. (2008) Subunit 1 of the prefoldin chaperone complex is required for lymphocyte development and function. J Immunol 181(1):476-84 |
| abstractText | 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. |
