AAV2 mediated subretinal Best1 gene delivery (under the control of VMD2 promoter) in a canine model (Best1 biallelic mutations) of autosomal recessive bestrophinopathy (ARB) showed sustained and long term reversal of lesions and micro detachment in the retina, restoration of ONL thickness to normal values, and improved structure at RPE-photoreceptor interface without any inflammatory response in the eye [229]. Here, BEST1 is linked to autosomal recessive bestrophinopathy.