Deletion of filamin A-interacting protein (FILIP) results in a weak grip strength and abnormal responses to nociceptive stimulation

Abstract

Filamin A-interacting protein (FILIP in mice, FILIP1 in humans) was first identified as a protein that negatively controls neuronal migration in rodents, and was subsequently demonstrated to be pivotal for the development of the neocortex. In the previous study, we generated FILIP knockout mice to investigate the in vivo functions of FILIP in cortical development. Since FILIP mRNA is widely expressed in the body, we systematically examined FILIP-knockout mice to determine the functions of FILIP throughout the body. Our results showed that FILIP-knockout mice exhibited weak grip strength and sensory abnormalities. Interestingly, we also found that FILIP was expressed in a subset of neurons in the dorsal root ganglion (DRG). Recent research has reported that FILIP1 mutations lead to severe neurological and musculoskeletal abnormalities, resulting in the proposal of a new disease entity, termed FILIP1opathy. It is expected that our FILIP-knockout mice could be used as a model for the pathological investigation of FILIP1opathy.