| First Author | Benavides F | Year | 2001 |
| Journal | Immunogenetics | Volume | 53 |
| Issue | 3 | Pages | 233-42 |
| PubMed ID | 11398968 | Mgi Jnum | J:69924 |
| Mgi Id | MGI:2135747 | Doi | 10.1007/s002510100320 |
| Citation | Benavides F, et al. (2001) The CD4 T cell-deficient mouse mutation nackt (nkt) involves a deletion in the cathepsin L (CtsI) gene. Immunogenetics 53(3):233-42 |
| abstractText | We recently reported a novel autosomal recessive mouse mutation designated nackt (nkt). Homozygous mutant mice have diffuse alopecia and a marked reduction in the proportion of CD4+ T cells in the thymus and peripheral lymphoid tissues. Here we show that the CD4 T-cell deficiency is due to a defect in the thymic microenvironment rather than the hematopoietic compartment. Furthermore, we identified the molecular basis of the mutant phenotype by demonstrating that the nkt mutation represents a 118-bp deletion of the cathepsin L (Ctsl) gene which is required for degradation of the invariant chain, a critical chaperone for major histocompatibility complex class II molecules. This finding explains the similarities in skin and immune defects observed in nkt/nkt and Ctsl -/- mice. The data reported here provide further in vivo evidence that the lysosomal cysteine protease cathepsin L plays a critical role in CD4+ T-cell selection in the thymus. |
