Ectopic PLAG1 induces muscular dystrophy in the mouse

Aguayo, Juan Shugert; Shelton, John M.; Wang, Tan; Rakheja, Dinesh; Cai, Chunyu; Shalaby, Ahmed; Lee, Jeon; Iannaccone, Susan T.; Xu, Lin; Chen, Kenneth; Burns, Dennis K.; Zheng, Yanbin

doi:10.1016/j.bbrc.2023.05.006

Cited by 1 publication

(1 citation statement)

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“…In addition to known roles in cancer, PLAG1 is also a critical developmental factor. PLAG1 is expressed in multiple embryonic cell lineages, with expression declining in most tissues postnatally [ 7 – 10 ]. Mice with a germline deletion of Plag1 ( Plag1 −/− ) presented with pronounced growth retardation, first detectable at embryonic day 11.5 (E11.5) and continuing throughout foetal development; by post-natal day 21 Plag1 −/− mice weigh 50% less than wildtype (WT) littermates [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

The Role of PLAG1 in Mouse Brain Development and Neurogenesis

Gasperoni,

Tran,

Grommen

et al. 2024

Mol Neurobiol

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The pleomorphic adenoma gene 1 (Plag1) is a transcription factor involved in the regulation of growth and cellular proliferation. Here, we report the spatial distribution and functional implications of PLAG1 expression in the adult mouse brain. We identified Plag1 promoter-dependent β-galactosidase expression in various brain structures, including the hippocampus, cortex, choroid plexus, subcommisural organ, ependymal cells lining the third ventricle, medial and lateral habenulae and amygdala. We noted striking spatial-restriction of PLAG1 within the cornu ammonis (CA1) region of the hippocampus and layer-specific cortical expression, with abundant expression noted in all layers except layer 5. Furthermore, our study delved into the role of PLAG1 in neurodevelopment, focusing on its impact on neural stem/progenitor cell proliferation. Loss of Plag1 resulted in reduced proliferation and decreased production of neocortical progenitors in vivo, although ex vivo neurosphere experiments revealed no cell-intrinsic defects in the proliferative or neurogenic capacity of Plag1-deficient neural progenitors. Lastly, we explored potential target genes of PLAG1 in the cortex, identifying that Neurogenin 2 (Ngn2) was significantly downregulated in Plag1-deficient mice. In summary, our study provides novel insights into the spatial distribution of PLAG1 expression in the adult mouse brain and its potential role in neurodevelopment. These findings expand our understanding of the functional significance of PLAG1 within the brain, with potential implications for neurodevelopmental disorders and therapeutic interventions.

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Section: Introductionmentioning

confidence: 99%