Mechanisms of Photoreceptor Death in Retinal Degenerations

Exclusion of five subunits of cGMP phosphodiesterase in Leber's congenital amaurosis

“…It is worth remembering that an increased cGMP level has been found in the photoreceptor membranes in all retinal dystrophies and apoptotic photoreceptor cell death has been documented in retinal degeneration (Adler 1996). Conversely, the LCA1 mutation, which is expected to cause a lack of cGMP in the retina, did not trigger apoptosis in a 16-month-old Japanese LCA girl.…”

Exclusion of five subunits of cGMP phosphodiesterase in Leber's congenital amaurosis

“…It is worth remembering that an increased cGMP level has been found in the photoreceptor membranes in all retinal dystrophies and apoptotic photoreceptor cell death has been documented in retinal degeneration (Adler 1996). Conversely, the LCA1 mutation, which is expected to cause a lack of cGMP in the retina, did not trigger apoptosis in a 16-month-old Japanese LCA girl.…”

A gradient of basic fibroblast growth factor in rod photoreceptors in the normal human retina

“…However, the previous studies did not utilize specific immunomarkcrs to identify the bFGF-containing cells and regional differences in bFGF were not noted. Previous studies demonstrated bFGF and its mRNA in the photoreceptor layer of animal retinas, including goldfish (Raymond et al, 1992), rodents (Noji et al, 1990;Connolly et al, 1992;Gao & Hollyfield, 1995, 1996Wen et al, 1995), and monkey (Kitaoka et al, 1994). A possible explanation for the latter differences is the use of human retinas with different postmortem times and conditions of fixation.…”

“…The ability of cones to survive longer than rods in the macula may in part be due to the causative mutation, which in some RP patients is rod-specific (e.g. However, most RP patients eventually lose cone cells, even in cases caused by mutant rod-specific genes, and it has been suggested that survival of the cones may depend on a product derived from the rods (Huang et al, 1993;Milam, 1993;Adler, 1996;Milam et al, 1996). acidic FGF or FGF-5 (Kitaoka et al, 1994)].…”

“…acidic FGF or FGF-5 (Kitaoka et al, 1994)]. However, most RP patients eventually lose cone cells, even in cases caused by mutant rod-specific genes, and it has been suggested that survival of the cones may depend on a product derived from the rods (Huang et al, 1993;Milam, 1993;Adler, 1996;Milam et al, 1996). Although bFGF lacks a signal peptide, there is evidence that it can be exported to the cell surface and act on nearby cells in a paracrine fashion (Baird, 1994).…”

Abnormal fundus autofluorescence in relation to retinal function in patients with retinitis pigmentosa

“…RPE changes in RP are secondary phenomena and result from increased metabolic load for the RPE due to photoreceptor apoptosis [1,29]. Accumulation of lipofuscin and therefore increased AF intensity is caused by faster turnover of photoreceptors due to genetic causes or through disturbed and ineffective disintegration of their remnants in the RPE [15,24].…”

Exclusion of five subunits of cGMP phosphodiesterase in Leber's congenital amaurosis

“…It is worth remembering that an increased cGMP level has been found in the photoreceptor membranes in all retinal dystrophies and apoptotic photoreceptor cell death has been documented in retinal degeneration (Adler 1996). Conversely, the LCA1 mutation, which is expected to cause a lack of cGMP in the retina, did not trigger apoptosis in a 16-month-old Japanese LCA girl.…”

A gradient of basic fibroblast growth factor in rod photoreceptors in the normal human retina

“…However, the previous studies did not utilize specific immunomarkcrs to identify the bFGF-containing cells and regional differences in bFGF were not noted. Previous studies demonstrated bFGF and its mRNA in the photoreceptor layer of animal retinas, including goldfish (Raymond et al, 1992), rodents (Noji et al, 1990;Connolly et al, 1992;Gao & Hollyfield, 1995, 1996Wen et al, 1995), and monkey (Kitaoka et al, 1994). A possible explanation for the latter differences is the use of human retinas with different postmortem times and conditions of fixation.…”

“…The ability of cones to survive longer than rods in the macula may in part be due to the causative mutation, which in some RP patients is rod-specific (e.g. However, most RP patients eventually lose cone cells, even in cases caused by mutant rod-specific genes, and it has been suggested that survival of the cones may depend on a product derived from the rods (Huang et al, 1993;Milam, 1993;Adler, 1996;Milam et al, 1996). acidic FGF or FGF-5 (Kitaoka et al, 1994)].…”

“…acidic FGF or FGF-5 (Kitaoka et al, 1994)]. However, most RP patients eventually lose cone cells, even in cases caused by mutant rod-specific genes, and it has been suggested that survival of the cones may depend on a product derived from the rods (Huang et al, 1993;Milam, 1993;Adler, 1996;Milam et al, 1996). Although bFGF lacks a signal peptide, there is evidence that it can be exported to the cell surface and act on nearby cells in a paracrine fashion (Baird, 1994).…”

Abnormal fundus autofluorescence in relation to retinal function in patients with retinitis pigmentosa

“…RPE changes in RP are secondary phenomena and result from increased metabolic load for the RPE due to photoreceptor apoptosis [1,29]. Accumulation of lipofuscin and therefore increased AF intensity is caused by faster turnover of photoreceptors due to genetic causes or through disturbed and ineffective disintegration of their remnants in the RPE [15,24].…”

Exclusion of five subunits of cGMP phosphodiesterase in Leber's congenital amaurosis

“…It is worth remembering that an increased cGMP level has been found in the photoreceptor membranes in all retinal dystrophies and apoptotic photoreceptor cell death has been documented in retinal degeneration (Adler 1996). Conversely, the LCA1 mutation, which is expected to cause a lack of cGMP in the retina, did not trigger apoptosis in a 16-month-old Japanese LCA girl.…”

A gradient of basic fibroblast growth factor in rod photoreceptors in the normal human retina

“…However, the previous studies did not utilize specific immunomarkcrs to identify the bFGF-containing cells and regional differences in bFGF were not noted. Previous studies demonstrated bFGF and its mRNA in the photoreceptor layer of animal retinas, including goldfish (Raymond et al, 1992), rodents (Noji et al, 1990;Connolly et al, 1992;Gao & Hollyfield, 1995, 1996Wen et al, 1995), and monkey (Kitaoka et al, 1994). A possible explanation for the latter differences is the use of human retinas with different postmortem times and conditions of fixation.…”

“…The ability of cones to survive longer than rods in the macula may in part be due to the causative mutation, which in some RP patients is rod-specific (e.g. However, most RP patients eventually lose cone cells, even in cases caused by mutant rod-specific genes, and it has been suggested that survival of the cones may depend on a product derived from the rods (Huang et al, 1993;Milam, 1993;Adler, 1996;Milam et al, 1996). acidic FGF or FGF-5 (Kitaoka et al, 1994)].…”

“…acidic FGF or FGF-5 (Kitaoka et al, 1994)]. However, most RP patients eventually lose cone cells, even in cases caused by mutant rod-specific genes, and it has been suggested that survival of the cones may depend on a product derived from the rods (Huang et al, 1993;Milam, 1993;Adler, 1996;Milam et al, 1996). Although bFGF lacks a signal peptide, there is evidence that it can be exported to the cell surface and act on nearby cells in a paracrine fashion (Baird, 1994).…”

Abnormal fundus autofluorescence in relation to retinal function in patients with retinitis pigmentosa

“…RPE changes in RP are secondary phenomena and result from increased metabolic load for the RPE due to photoreceptor apoptosis [1,29]. Accumulation of lipofuscin and therefore increased AF intensity is caused by faster turnover of photoreceptors due to genetic causes or through disturbed and ineffective disintegration of their remnants in the RPE [15,24].…”