Abstract
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 language | English |
|---|---|
| Pages (from-to) | 476-484 |
| Number of pages | 9 |
| Journal | Journal of Immunology |
| Volume | 181 |
| Issue number | 1 |
| State | Published - Dec 1 2008 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Immunology
Cite this
Subunit 1 of the Prefoldin chaperone complex is required for lymphocyte development and function. / Cao, Shang; Carlesso, Gianluca; Osipovich, Anna B.; Llanes, Joan; Lin, Qing; Hoek, Kristen L.; Khan, Wasif; Ruley, H. Earl.
In: Journal of Immunology, Vol. 181, No. 1, 01.12.2008, p. 476-484.Research output: Contribution to journal › Article
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TY - JOUR
T1 - Subunit 1 of the Prefoldin chaperone complex is required for lymphocyte development and function
AU - Cao, Shang
AU - Carlesso, Gianluca
AU - Osipovich, Anna B.
AU - Llanes, Joan
AU - Lin, Qing
AU - Hoek, Kristen L.
AU - Khan, Wasif
AU - Ruley, H. Earl
PY - 2008/12/1
Y1 - 2008/12/1
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=47949127947&partnerID=8YFLogxK
M3 - Article
C2 - 18566413
AN - SCOPUS:47949127947
VL - 181
SP - 476
EP - 484
JO - Journal of Immunology
JF - Journal of Immunology
SN - 0022-1767
IS - 1
ER -