2015
DOI: 10.1016/j.mod.2014.11.004
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Chronic up-regulation of the SHH pathway normalizes some developmental effects of trisomy in Ts65Dn mice

Abstract: Down Syndrome (DS) is a highly complex developmental genetic disorder caused by trisomy for human chromosome 21 (Hsa21). All individuals with DS exhibit some degree of brain structural changes and cognitive impairment; mouse models such as Ts65Dn have been instrumental in understanding the underlying mechanisms. Several phenotypes of DS might arise from a reduced response of trisomic cells to the Sonic Hedgehog (SHH) growth factor. If all trisomic cells show a similar reduced response to SHH, then up-regulatio… Show more

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Cited by 15 publications
(20 citation statements)
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References 50 publications
(104 reference statements)
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“…We crossed Ts65Dn and Ptch1 tm1Mps/+ mice to generate four genotypes: Ts65Dn mice (Ts;WT); Ts65Dn mice haploinsufficient for Ptch1 (Ts; Ptch +/− ); euploid control (Eu;WT); and euploid haploinsufficient for Ptch1 allele (Eu; Ptch +/− ) (Table ) (Dutka et al, ). Three‐dimensional coordinates of biological landmarks measured on the skulls of adult mice were collected from micro‐computed tomography (micro‐CT) images and analyzed (Fig.…”
Section: Resultsmentioning
confidence: 99%
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“…We crossed Ts65Dn and Ptch1 tm1Mps/+ mice to generate four genotypes: Ts65Dn mice (Ts;WT); Ts65Dn mice haploinsufficient for Ptch1 (Ts; Ptch +/− ); euploid control (Eu;WT); and euploid haploinsufficient for Ptch1 allele (Eu; Ptch +/− ) (Table ) (Dutka et al, ). Three‐dimensional coordinates of biological landmarks measured on the skulls of adult mice were collected from micro‐computed tomography (micro‐CT) images and analyzed (Fig.…”
Section: Resultsmentioning
confidence: 99%
“…The multivariate regression analysis (Fig. ) does capture some size differences between the Eu;WT and Eu; Ptch +/− mice, with the latter showing a marked increase in size (on the higher end of the centroid size axis), suggesting that haploinsufficiency for Ptch1 does, to an extent, affect craniofacial size in the euploid mice (Dutka et al, ). This analysis also indicates that size related changes in cranial shape (i.e., allometry) are different between the euploid and trisomic mice.…”
Section: Discussionmentioning
confidence: 99%
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“…However, a deficit in a cerebellum-dependent motor learning task (phase reversal adaptation and consolidation of the vestibulo-ocular reflex) is not ameliorated by postnatal SAG treatment in Ts65Dn mice, demonstrating brain region/neuronal circuit-dependent effects of Shh signaling in this DS model [53]. Consistent with complex roles for Shh in DS are data showing that crossing of Ts65Dn mice with mice with reduced Ptch expression (which increases Shh signaling) normalizes some, but not all, of the brain structural and behavioral phenotypes in the DS mice [54]. …”
Section: Shh Signaling and Neurological Disordersmentioning
confidence: 98%