BEST1 and autosomal recessive bestrophinopathy: The BEST1 gene encodes the bestrophin-1 protein that localizes to the RPE.[21, 22] Bestrophinopathies caused by BEST1 gene mutations are found to be associated with reduced AL, hyperopia and a high incidence of ACG.[6, 8, 9, 23, 24] Up to 50% of ARB patients are at risk of angle closure glaucoma developing as a result of narrow anterior chamber angles.[6, 14] But the role of bestrophin-1 in the development of ACG has not been elucidated.