About   Help   FAQ
Phenotypes Associated with This Genotype
Genotype
MGI:2665401
Allelic
Composition
Fmr1tm1Cgr/Fmr1tm1Cgr
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Fmr1tm1Cgr mutation (5 available); any Fmr1 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
behavior/neurological
• in all eye blink conditioning training session the percentage and peak amplitudes of the startle responses were higher

nervous system
• 60.4% decrease in neuronal differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)
• 74.9% increase in astrocyte differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)
• exogenously expressed wild-type gene, but not mutant (I304N) rescues both the neuronal and the astrocyte differentiation deficits in homozygous mutant cells
• reduced (10.4% ) neuronal differentiation but greater (75.7%) glial differentiation in aNPCs residing in the DG compared with wild-type mice
• increased proliferation of isolated aNPCs from both the forebrain and DG of adult homozygous mice
• 11% more cells in mitotic (G2/M) phase compared with wild-type controls
• increased proliferation of both stem and progenitor cells in the DG and subventricular zone of mutant mice
• normal proliferation of astrocytes in the DG of mutant mice
• increased volume of the dentate gyrus (DG) in mutant mice
• the percentage of single climbing fiber innervation is increased, the length of spine heads and necks is increased, and spines are more irregular
• induction of long term depression in Purkinje cells is significantly enhanced when stimulating parallel fibers

cellular
• 60.4% decrease in neuronal differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)
• 74.9% increase in astrocyte differentiation compared with wild-type isolated adult neural progenitor/stem cells (aNPCs)
• exogenously expressed wild-type gene, but not mutant (I304N) rescues both the neuronal and the astrocyte differentiation deficits in homozygous mutant cells
• reduced (10.4% ) neuronal differentiation but greater (75.7%) glial differentiation in aNPCs residing in the DG compared with wild-type mice
• increased proliferation of isolated aNPCs from both the forebrain and DG of adult homozygous mice
• 11% more cells in mitotic (G2/M) phase compared with wild-type controls
• increased proliferation of both stem and progenitor cells in the DG and subventricular zone of mutant mice
• normal proliferation of astrocytes in the DG of mutant mice

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
fragile X syndrome DOID:14261 OMIM:300624
J:101021


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources
Funding Information
Warranty Disclaimer, Privacy Notice, Licensing, & Copyright
Send questions and comments to User Support.
last database update
12/10/2024
MGI 6.24
The Jackson Laboratory