nervous system
• male mice with 102 to 110 CGG repeats exhibit ubiquitin positive intranuclear neuronal inclusions
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Allele Symbol Allele Name Allele ID |
Fmr1tm2Cgr targeted mutation 2, Ben Oostra MGI:2451086 |
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Summary |
2 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• male mice with 102 to 110 CGG repeats exhibit ubiquitin positive intranuclear neuronal inclusions
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• at 72 weeks, male mice (106 to 123 CGG repeats) in an open field test travel shorter distances and enter the center circle fewer times than wild-type mice
• however, activity and exploration levels are normal
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• at 52 weeks, male mice (106 to 123 CGG repeats) in a Morris water maze exhibit impaired visual-spatial learning and memory compared to wild-type mice with increased escape latency and path length
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• male mice (106 to 123 CGG repeats) exhibit decreased performance on a rotarod as they age unlike wild-type mice
• at 52 and 72 weeks, male mice (106 to 123 CGG repeats) exhibit reduced latency to fall on a rotarod test compared to at 20 weeks of age
• at 72 weeks, male mice (106 to 123 CGG repeats) exhibit a lower number of segments crossed on a stationary beam compared to wild-type mice
• however, mice perform as wild-type mice in a gait test
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• at 52 weeks, male mice (106 to 123 CGG repeats) exhibit decreased latency to first fall in a wire suspension test and fall more often than wild-type mice
• however, performance in a wire suspension test is normal at 20 and 72 weeks of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
fragile X-associated tremor/ataxia syndrome | DOID:0050879 |
OMIM:300623 |
J:104445 |
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/19/2024 MGI 6.24 |
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