Membrane integral NOTCH2-associated receptor 2 (MINAR2)

Membrane integral NOTCH2-associated receptor 2 (MINAR2) is a membrane protein essential for normal hearing in humans and mice, encoded by the MINAR2 gene[1][2]. MINAR2 is highly expressed in the cochlear hair cells, spiral ganglia, spiral limbus, and stria vascularis of the inner ear, where it supports proper auditory function without being required for hair cell survival during early development. Loss-of-function variants in MINAR2 cause autosomal recessive, nonsyndromic sensorineural hearing loss due to progressive disruption of cochlear hair cell synapses and stereocilia organization. Mechanistically, MINAR2 acts as a negative regulator of NOTCH2 signaling, downregulates VEGFA, and inhibits mTOR and MAP kinase pathways. No drugs are currently known to target MINAR2, but its restricted function and preserved hair cells in mutant animals make it a candidate for genetic therapies for hereditary deafness[1][2].

Other names

Major intrinsically disordered NOTCH2-binding receptor 1-likeMINAR2KIAA1024LDFNB120UPF0258 protein KIAA1024-likeMembrane integral NOTCH2 associated receptor 2Major intrinsically disordered NOTCH2-associated receptor 2Major intrinsically disordered Notch2-binding receptor 1-like

No drugs targeting MINAR2 are currently described; mechanistic studies suggest its activity involves suppression of NOTCH2, VEGF, mTOR, and MAP kinase (ERK1/2) pathways[1][2].