Additionally, mutations in genes involved in the transport of surfactant lipids within lamellar bodies, such as ABCA3 (ATP binding cassette subfamily A member 3), and in regulatory genes such as NKX2-1 (encoding the thyroid transcription factor 1, TTF1), which controls the transcription of surfactant proteins and ABCA3, have also been identified [8]; therefore, disruptions in the factors responsible for surfactant system homeostasis are now recognized as monogenic causes of interstitial lung disease [54]. Here, NKX2-1 is linked to interstitial lung disease.