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Erin Paul Marissa Kielbasinski John M. Sedivy Carlos Murga-Zamalloa Hemant Khanna Jan E. Klysik 《Transgenic research》2010,19(4):691-701
Supv3L1 is an evolutionarily conserved helicase that plays a critical role in the mitochondrial RNA surveillance and degradation
machinery. Conditional ablation of Supv3L1 in adult mice leads to premature aging phenotypes including loss of muscle mass and adipose tissue and severe skin abnormalities.
To get insights into the spatial and temporal expression of Supv3L1 in the mouse, we generated knock-in and transgenic strains
in which an EGFP reporter was placed under control of the Supv3L1 native promoter. During development, expression of Supv3L1 begins at the blastocyst stage, becomes widespread and strong
in all fetal tissues and cell types, and continues during postnatal growth. In mature animals reporter expression is only
slightly diminished in most tissues and continues to be highly expressed in the brain, peripheral sensory organs, and testis.
Together, these data confirm that Supv3L1 is an important developmentally regulated gene, which continues to be expressed
in all mature tissues, particularly the rapidly proliferating cells of testes, but also in the brain and sensory organs. The
transgenic mice and cell lines derived from them constitute a valuable tool for the examination of the spatial and temporal
aspects of Supv3L1 promoter activity, and should facilitate future screens for small molecules that regulate Supv3L1 expression. 相似文献
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Parallelism and convergence in anuran fangs 总被引:1,自引:0,他引:1
The anuran lower jaw is composed of three pairs of bones: dentaries, angulosplenials and mentomeckelians. Although the lower jaw is toothless, except in Gastrotheca guentheri , enlarged fangs or odontoids have evolved at least four times independently in some myobatrachids, hylids, ranids and leptodactylids through both parallel and convergent evolutionary events. Fangs seem to represent the single best design solution to enable an anuran to inflict a bite-like wound, but the biological role of biting varies among species. Fangs are projections of the dentaries in ranids, but in the hylid frog Hemiphractus and in ceratophryine leptodactylids, they form a sinosteotic unit with the dentaries and mentomeckelians. Comparisons of morphology, behaviour and diet among frog taxa with enlarged fangs reveal that the fangs may be the result of either sexual or natural selection. Those fangs that evolved in response to sexual selection seem to be relatively larger than those that resulted of natural selection. 相似文献
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Jennie C L Roy Antonia Vitalo Marissa A Andrew Eduarda Mota-Silva Marina Kovalenko Zoe Burch Anh M Nhu Paula E Cohen Ed Grabczyk Vanessa
C Wheeler Ricardo Mouro
Pinto 《Nucleic acids research》2021,49(7):3907
Somatic expansion of the CAG repeat tract that causes Huntington''s disease (HD) is thought to contribute to the rate of disease pathogenesis. Therefore, factors influencing repeat expansion are potential therapeutic targets. Genes in the DNA mismatch repair pathway are critical drivers of somatic expansion in HD mouse models. Here, we have tested, using genetic and pharmacological approaches, the role of the endonuclease domain of the mismatch repair protein MLH3 in somatic CAG expansion in HD mice and patient cells. A point mutation in the MLH3 endonuclease domain completely eliminated CAG expansion in the brain and peripheral tissues of a HD knock-in mouse model (HttQ111). To test whether the MLH3 endonuclease could be manipulated pharmacologically, we delivered splice switching oligonucleotides in mice to redirect Mlh3 splicing to exclude the endonuclease domain. Splice redirection to an isoform lacking the endonuclease domain was associated with reduced CAG expansion. Finally, CAG expansion in HD patient-derived primary fibroblasts was also significantly reduced by redirecting MLH3 splicing to the endogenous endonuclease domain-lacking isoform. These data indicate the potential of targeting the MLH3 endonuclease domain to slow somatic CAG repeat expansion in HD, a therapeutic strategy that may be applicable across multiple repeat expansion disorders. 相似文献
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Yongguang Yang Marissa Leonard Zhenhua Luo Syn Yeo Gregory Bick Mingang Hao Chunmiao Cai Mahmoud Charif Jiang Wang Jun-Lin Guan Elyse E. Lower Xiaoting Zhang 《Cell reports》2021,34(10):108822
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