Mouse Genome Informatics
hm
    Faslpr/Faslpr
C3.MRL-Faslpr
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
homeostasis/metabolism
• stimulation with concanavalin A does not induce cells to produce Il2

immune system
• at 2 months, glandular inflammation is neglible; at 5 months, nearly all mice exhibit lacrimal gland inflammation covering a larger area than in mutants at 2 months or controls at 5 months
• inflammation correlates with age, immune complex level and spleen weight; antinuclear antibody level correlation is greater than probability cutoff; controls do not show correlations with these factors and gland inflammation
• inflammatory infiltrates consist of mononuclear cells and occurs in a periductal or perivascular pattern
• scattered lobular atrophy with loss of secretory elements is seen in glands with multifocal infiltrates
• lymph node cells (T cell origin) are abnormal; cells are Ly-2-/L3T4-/surface Ig-
• cells do not generate CTL in response to stimulation with alloantigens
• cells do not proliferate in response to stimulation with alloantigens
• stimulation with concanavalin A does not induce cells to produce Il2

endocrine/exocrine glands
N
• submandibular gland inflammation is observed in most mice at 5 months, but differences compared to wild-type are not significant (J:1028)
• no parotid gland inflammation is observed and only 1 animal showed sublingual gland inflammation at 5 months (J:1028)
• in inflamed lacrimal glands, lobular boundaries are preserved with preservation of interlobular septae; lobular atrophy occurs with preservation of ductal epithelium; widely dilated ducts indicate that ductal obstruction is not observed (J:1028)
• at 2 months, glandular inflammation is neglible; at 5 months, nearly all mice exhibit lacrimal gland inflammation covering a larger area than in mutants at 2 months or controls at 5 months
• inflammation correlates with age, immune complex level and spleen weight; antinuclear antibody level correlation is greater than probability cutoff; controls do not show correlations with these factors and gland inflammation
• inflammatory infiltrates consist of mononuclear cells and occurs in a periductal or perivascular pattern
• scattered lobular atrophy with loss of secretory elements is seen in glands with multifocal infiltrates

hematopoietic system
• lymph node cells (T cell origin) are abnormal; cells are Ly-2-/L3T4-/surface Ig-
• cells do not generate CTL in response to stimulation with alloantigens
• cells do not proliferate in response to stimulation with alloantigens

vision/eye
• at 2 months, glandular inflammation is neglible; at 5 months, nearly all mice exhibit lacrimal gland inflammation covering a larger area than in mutants at 2 months or controls at 5 months
• inflammation correlates with age, immune complex level and spleen weight; antinuclear antibody level correlation is greater than probability cutoff; controls do not show correlations with these factors and gland inflammation
• inflammatory infiltrates consist of mononuclear cells and occurs in a periductal or perivascular pattern
• scattered lobular atrophy with loss of secretory elements is seen in glands with multifocal infiltrates

digestive/alimentary system

cellular
• unlike wild-type vaginal cells, vaginal cells derived from mutant mice do not undergo apoptosis after treatment with agonistic anti-mouse Fas antibody or mouse recombinant TNF antibody
• neuron viability is comparable to wild-type when grown in absence of Abeta or if treated with KCN which induces necrotic cell death
• very low levels of apoptosis (15%) compared to wild-type (60%) are seen when cortical neurons are treated with Abeta25-35 or Abeta1-40 peptides

nervous system
• neuron viability is comparable to wild-type when grown in absence of Abeta or if treated with KCN which induces necrotic cell death
• very low levels of apoptosis (15%) compared to wild-type (60%) are seen when cortical neurons are treated with Abeta25-35 or Abeta1-40 peptides

reproductive system
• at 2 days after estrogen deprivation induced by gonadectomy, mutant females show no vaginal regression (measured by a decrease in vaginal organ weight), indicating no Fas-mediated vaginal cell death, in contrast to wild-type females that show >50% decrease in vaginal organ weight (J:114219)

Mouse Models of Human Disease
OMIM IDRef(s)
Sjogren Syndrome 270150 J:1028