Interphotoreceptor matrix proteoglycan-2 is part of an extracellular complex occupying the interface between photoreceptors and the retinal pigment epithelium in the fundus of the eye.[supplied by OMIM
Full Name
interphotoreceptor matrix proteoglycan 2
Function
Chondroitin sulfate- and hyaluronan-binding proteoglycan involved in the organization of interphotoreceptor matrix; may participate in the maturation and maintenance of the light-sensitive photoreceptor outer segment. Binds heparin.
Biological Process
Extracellular matrix organizationIEA:Ensembl
Protein localizationIEA:Ensembl
Retina morphogenesis in camera-type eyeIEA:Ensembl
Visual perceptionManual Assertion Based On ExperimentTAS:UniProtKB
Cellular Location
Photoreceptor outer segment membrane; Photoreceptor inner segment membrane; Secreted, extracellular space, extracellular matrix, interphotoreceptor matrix
Involvement in disease
Retinitis pigmentosa 56 (RP56):
A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.
Macular dystrophy, vitelliform, 5 (VMD5):
A form of macular dystrophy, a retinal disease in which various forms of deposits, pigmentary changes, and atrophic lesions are observed in the macula lutea. Vitelliform macular dystrophies are characterized by yellow, lipofuscin-containing deposits, usually localized at the center of the macula. VMD5 features include late-onset moderate visual impairment and preservation of retinal pigment epithelium reflectivity.
Topology
Extracellular: 23-1099
Helical: 1100-1120
Cytoplasmic: 1121-1241
PTM
Highly glycosylated (N- and O-linked carbohydrates).