Related Experiment Video
Updated: Jun 13, 2026
07:44
Preparation of 3D Decellularized Matrices from Fetal Mouse Skeletal Muscle for Cell Culture
Published on: March 3, 2023
Cell-extrinsic defective lymphocyte development in Lmna(-/-) mice.
J Scott Hale1, Richard L Frock, Sara A Mamman
1Department of Immunology, University of Washington, Seattle, Washington, United States of America.
Plos One
|April 21, 2010
Summary
Mutations in the LMNA gene cause laminopathies. In Lmna(-/-) mice, lymphocyte development defects are not due to direct lamin loss but likely indirect damage from muscle dystrophies.
Area of Science:
- Cell Biology
- Immunology
- Genetics
Background:
- Mutations in the LMNA gene cause laminopathies, a group of human diseases.
- Lmna(-/-) mice exhibit growth retardation and tissue defects mimicking human laminopathies, including smaller lymphoid organs.
Purpose of the Study:
- To investigate the correlation between altered lymphoid organ sizes and specific defects in lymphocyte development in Lmna(-/-) mice.
Main Methods:
- Bone marrow reconstitution experiments in wild-type recipients.
- Thymus lobe transplantation into syngeneic wild-type recipients.
Main Results:
- Lmna(-/-) mice show age-dependent defects in T and B cell development, correlating with runting.
- Bone marrow from Lmna(-/-) mice can restore normal T and B cell development in irradiated wild-type recipients.
- Transplanted Lmna(-/-) thymus lobes support normal thymocyte development in wild-type hosts.
Conclusions:
- Severe lymphocyte development defects in Lmna(-/-) mice do not stem from direct loss of A-type lamin function in lymphocytes or thymic stroma.
- Immune defects are likely an indirect consequence of prolonged stress from striated muscle dystrophies in Lmna(-/-) mice.
