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

Shang Cao, Gianluca Carlesso, Anna B. Osipovich, Joan Llanes, Qing Lin, Kristen L. Hoek, Wasif N. Khan, H. Earl Ruley

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


Prefoldin is a hexameric chaperone that facilitates posttranslational folding of actins and other cytoskeletal proteins by the Tcpl-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 thynius. 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.

Original languageEnglish (US)
Pages (from-to)476-484
Number of pages9
JournalJournal of Immunology
Issue number1
StatePublished - 2008
Externally publishedYes

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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