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<i>Nf1</i> deletion results in depletion of the <i>Lhx6</i> transcription factor and a specific loss of parvalbumin <sup>+</sup> cortical interneurons

Kartik Angara, Emily Ling-Lin Pai, Stephanie M. Bilinovich, April M Stafford, Julie Nguyen, Katie X. Li, Anirban Paul, John L.R. Rubenstein, Daniel Vogt

2020Proceedings of the National Academy of Sciences30 citationsDOIOpen Access PDF

Abstract

Significance NF1 is a monogenic disorder caused by mutations in the NF1 gene. While it affects multiple organs and tissues, how brain symptoms arise has been complicated to uncover. Since brain glial and inhibitory neuron cells have been implicated in NF1, we conditionally deleted the mouse Nf1 gene in progenitors that give rise to both of these cell types. We found that glial cells were impacted when both copies of Nf1 were deleted, while properties of a specific group of inhibitory neurons were altered when either one or both copies of Nf1 were deleted. These data demonstrate phenotypes that may contribute to NF1 and uncover roles for the Nf1 gene in brain development.

Topics & Concepts

ParvalbuminBiologyPhenotypeGeneTranscription factorNeuronGanglionic eminenceGeneticsInhibitory postsynaptic potentialNeuroscienceCell biologyEmbryonic stem cellGenetics and Neurodevelopmental DisordersChromatin Remodeling and CancerCongenital heart defects research
<i>Nf1</i> deletion results in depletion of the <i>Lhx6</i> transcription factor and a specific loss of parvalbumin <sup>+</sup> cortical interneurons | Litcius