An early decrease in interphotoreceptor retinoid-binding protein gene expression in Abyssinian cats homozygous for hereditary rod-cone degeneration.
Levels of interphotoreceptor retinoid-binding protein (IRBP) protein and message in retinas of Abyssinian cats homozygous for progressive rod-cone degeneration were determined at early ages, well before the onset of clinical retinal degeneration. IRBP gene expression was assessed by immunochemical quantitation of IRBP protein, and by Northern blotting and slot-blotting of total RNA using a human IRBP cDNA probe. Morphology was assessed by electron microscopy and immunocytochemistry. Levels of both IRBP protein and message in affected Abyssinian cat retinas were significantly reduced below normal as early as 4 weeks of age at the earliest stage of retinal disorientation. Opsin mRNA was more abundant in affected Abyssinian cat retinas than in control retinas. This was at least 1 year before the onset of clinical symptoms. The reduction in IRBP gene expression to levels significantly below normal well before the onset of retinal degeneration in affected Abyssinian cat retinas indicates that this represents a primary defect or at least an early problem that could itself cause adverse effects.
Wiggert
B
B
National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892.
van Veen
T
T
Kutty
G
G
Lee
L
L
Nickerson
J
J
Si
J S
JS
Nilsson
S E
SE
Chader
G J
GJ
Narfström
K
K
D000818:Animals; D015152:Blotting, Northern; D002415:Cats / Q000235:genetics*; D004247:DNA / Q000032:analysis / Q000235:genetics; D005136:Eye Proteins; D015870:Gene Expression; D006720:Homozygote; D007150:Immunohistochemistry; D008854:Microscopy, Electron; D010786:Photoreceptor Cells / Q000737:chemistry / Q000648:ultrastructure; D012174:Retinitis Pigmentosa / Q000235:genetics* / Q000473:pathology; D012177:Retinol-Binding Proteins / Q000032:analysis / Q000235:genetics*; D000099214:Retinol-Binding Proteins, Interstitial
D004247:DNA; D005136:Eye Proteins; D012177:Retinol-Binding Proteins; D000099214:Retinol-Binding Proteins, Interstitial