The ZAP-70 allelic series and Lat mouse models bear a much closer resemblance to these relatively rare clinical entities than to common polygenic rheumatic diseases such as RA and SLE.
Accumulation of the combination of unsaturated 18 carbon FAEEs (such as ethyl oleate + linoleate + linolenate [OLL]) as well as the saturated 18 carbon stearate has been described in multiple alcohol-exposed organs, meconium, and other samples (such as hair) in guinea pig (Brien et al., 2006; Kulaga et al., 2009) and mouse models (Bearer et al., 1992).
However, the experiments in the human-synovium severe-combined-immunodeficiency-mouse model bear close resemblance to thuman-synovium severe-combined-immunodeficiency-mousebe expected to be affected.
Here, Trevor Williams and colleagues develop a new mouse model that bears mutations in the gene encoding transcription factor AP-2α (Tfassociatedsociated with human CL/P – and that shows full CL/P penetrance.
The Atxn1 knockin mouse model, which bears a 154 CAG repeat knocked into the murine Atxn1 locus, faithfully reproduces the SCA1 phenotype: progressive motor incoordination due to cerebellar degeneration, cognitive deficits, premature death, and degradation-resistant deposits (nuclear inclusions, or NIs) that contain mutant ATXN1 (Watase et al., 2002).
To address this, investigators from the laboratories of Rajkumar and Medina [ 1] have examined the effects of exogenous estrogen and progesterone in mouse models that bear lesions in clinically relevant pathways.
Scurfy, the mouse model of autoimmune IPEX syndrome, bears a loss-of-function mutation in Foxp3 that leads to multi-organ inflammation.
At the animal level, the mouse model generated by Hoki et al. which bears in frame deletion of exon 13 RecQ4 shows normal sensitivity to IR and UV irradiation [28].
Although the use of only one mouse model of AD is an important caveat to bear in mind, we believe that our results could pertain to a general mechanism of plaque growth.
25, 26 A mouse model of BRAF-V600E mutant melanoma, in which tumors bore close pathological and genetic similarity to those seen in humans, established this mutant as a driving event in melanomagenesis.
