Ca²⁺-binding protein 2 inhibits Ca²⁺-channel inactivation in mouse inner hair cells.

ABSTRACT: Ca²⁺-binding protein 2 (CaBP2) inhibits the inactivation of heterologously expressed voltage-gated Ca²⁺ channels of type 1.3 (Ca_V1.3) and is defective in human autosomal-recessive deafness 93 (DFNB93). Here, we report a newly identified mutation in CABP2 that causes a moderate hearing impairment likely via nonsense-mediated decay of CABP2-mRNA. To study the mechanism of hearing impairment resulting from CABP2 loss of function, we disrupted Cabp2 in mice (Cabp2^LacZ/LacZ ). CaBP2 was expressed by cochlear hair cells, preferentially in inner hair cells (IHCs), and was lacking from the postsynaptic spiral ganglion neurons (SGNs). Cabp2^LacZ/LacZ mice displayed intact cochlear amplification bu

SUBMITTER: Picher MM 

PROVIDER: S-EPMC5338518 | biostudies-literature | 2017 Feb

REPOSITORIES: biostudies-literature