2012
DOI: 10.4049/jimmunol.1100942
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Reduced Lymphocyte Longevity and Homeostatic Proliferation in Lamin B Receptor-Deficient Mice Results in Profound and Progressive Lymphopenia

Abstract: The lamin B receptor (LBR) is a highly unusual inner nuclear membrane protein with multiple functions. Reduced levels are associated with decreased neutrophil lobularity, whereas complete absence of LBR results in severe skeletal dysplasia and in utero/perinatal lethality. We describe a mouse pedigree, Lym3, with normal bone marrow and thymic development but profound and progressive lymphopenia particularly within the T cell compartment. This defect arises from a point mutation within the Lbr gene with only tr… Show more

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Cited by 13 publications
(11 citation statements)
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“…In marked contrast, the neutrophils of (NZW×B6.Lbr ic )F 1 mice displayed prominent alterations in nuclear morphology (Fig. 3A,B), which is consistent with the impairment in Lbr expression (Hoffmann et al, 2002; Shultz et al, 2003; Verhagen et al, 2012). The disruption in the nuclear morphology of neutrophils establishes the autosomal dominant alteration in nuclear structure, which is attributable to impaired Lbr expression in mice that inherited the Lbr ic mutation.…”
Section: Resultssupporting
confidence: 74%
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“…In marked contrast, the neutrophils of (NZW×B6.Lbr ic )F 1 mice displayed prominent alterations in nuclear morphology (Fig. 3A,B), which is consistent with the impairment in Lbr expression (Hoffmann et al, 2002; Shultz et al, 2003; Verhagen et al, 2012). The disruption in the nuclear morphology of neutrophils establishes the autosomal dominant alteration in nuclear structure, which is attributable to impaired Lbr expression in mice that inherited the Lbr ic mutation.…”
Section: Resultssupporting
confidence: 74%
“…2B). This is in contrast to the gradual reduction in spleen size in B6 mice with impaired Lbr expression (Verhagen et al, 2012). Whereas complete loss of Lbr expression causes runting (Shultz et al, 2003), the body weight of (NZW×B6.Lbr ic )F 1 mice did not differ from (NZW×B6)F 1 (Fig.…”
Section: Resultsmentioning
confidence: 61%
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“…Nevertheless, it is striking that mice lacking a fully functional LBR allele due to homozygous mutations at the ichthyosis locus ( ic/ic ) are viable, although these animals display numerous phenotypic abnormalities including alopecia, syndactyly and hydrocephalus as well as an increase in embryonic lethality ( Shultz et al, 2003 ). Growth defects were also observed in primary cells isolated from mouse models with mutations in the LBR gene ( Subramanian et al, 2012 ; Verhagen et al, 2012 ). Of note, defects in neutrophil maturation in ic/ic animals can be recued in vitro by expression of the LBR SRD ( Subramanian et al, 2012 ).…”
Section: Discussionmentioning
confidence: 99%