Subunit 1 of the prefoldin chaperone complex is required for lymphocyte development and function.

Cao S, Carlesso G, Osipovich AB, Llanes J, Lin Q, Hoek KL, Khan WN, Ruley HE
J Immunol. 2008 181 (1): 476-84

PMID: 18566413 · DOI:10.4049/jimmunol.181.1.476

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.

MeSH Terms (12)

Animals Base Sequence Bone Marrow Cell Differentiation Lymphocytes Mice Mice, Knockout Molecular Chaperones Molecular Sequence Data Protein Subunits Spleen Thymus Gland

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