IMPG2

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).