The cone matrix sheath in the normal and diseased retina: Cytochemical and biochemical studies of peanut agglutinin-binding proteins in cone and rod-cone degeneration

摘要: Abstract The fate of the cone-associated extracellular domain, or cone matrix sheath (CMS), was examined in two canine models hereditary retinal degeneration. diseases, which affect cones selectively (cd = degeneration), rods and temporally (prcd progressive rod-cone were biochemically (SDS-PAGE/lectin blots) cytochemically (light microscopy) using peanut agglutinin lectin (PNA) to label this domain associated structures. Most had disappeared adult cd retina. In remaining cones, PNA labeled ectopically located somata CMSs that present around severely diseased ones. Loss resulted background IPM loss pedicle-associated OPL. SDS-PAGE extracts showed all major classes lower molecular weight PNA-binding proteins present, but only 40- 60-kD bands remained prominent. Because selectivity mutation, suggests considerable heterogeneity within various size classifications glycoproteins. prcd, normal at a time when structurally disease limited rod outer segments. intact during degenerative phase disease, became compressed association with collapse narrowing photoreceptor layer; CMS labeling lost disappearance inner segment. biochemical results until 1·7 years age; thereafter, there decreased prominence bands. spatial severity, it not possible correlate cytochemical prcd.