Compensation between FOXP transcription factors maintains proper striatal function

Cell Rep. 2024 May 28;43(5):114257. doi: 10.1016/j.celrep.2024.114257. Epub 2024 May 17.

Abstract

Spiny projection neurons (SPNs) of the striatum are critical in integrating neurochemical information to coordinate motor and reward-based behavior. Mutations in the regulatory transcription factors expressed in SPNs can result in neurodevelopmental disorders (NDDs). Paralogous transcription factors Foxp1 and Foxp2, which are both expressed in the dopamine receptor 1 (D1) expressing SPNs, are known to have variants implicated in NDDs. Utilizing mice with a D1-SPN-specific loss of Foxp1, Foxp2, or both and a combination of behavior, electrophysiology, and cell-type-specific genomic analysis, loss of both genes results in impaired motor and social behavior as well as increased firing of the D1-SPNs. Differential gene expression analysis implicates genes involved in autism risk, electrophysiological properties, and neuronal development and function. Viral-mediated re-expression of Foxp1 into the double knockouts is sufficient to restore electrophysiological and behavioral deficits. These data indicate complementary roles between Foxp1 and Foxp2 in the D1-SPNs.

Keywords: CP: Molecular biology; CP: Neuroscience; FOXP1; FOXP2; autism; neurogenomics; spiny projection neurons; striatum.

MeSH terms

  • Animals
  • Corpus Striatum* / metabolism
  • Forkhead Transcription Factors* / genetics
  • Forkhead Transcription Factors* / metabolism
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Neurons / metabolism
  • Receptors, Dopamine D1 / genetics
  • Receptors, Dopamine D1 / metabolism
  • Repressor Proteins / genetics
  • Repressor Proteins / metabolism
  • Social Behavior

Substances

  • Forkhead Transcription Factors
  • Foxp1 protein, mouse
  • Repressor Proteins
  • Foxp2 protein, mouse
  • Receptors, Dopamine D1