Fatty acid metabolism in normal miniature poodles and those affected with progressive rod-cone degeneration (prcd).
It is possible that a genetic defect observed in the prcd poodle involves the abnormality of an enzyme which functions in phospholipid or lipoprotein metabolism. In our studies thus far, we have been unable to detect any defect in retinal phospholipid biosynthesis, but we have noted a decrease in plasma levels of 22:6w3 which may be a result of an enzyme defect in liver biosynthesis of 22:6w3 from its dietary precursor, linolenic acid, or some defect in the blood lipoprotein transport of this essential fatty acid. If 22:6w3 is essential to the normal elaboration and functioning of the photoreceptor outer segment, it is possible that decreased access to this fatty acid due to lower blood levels of 22:6w3 could cause photoreceptor abnormalities. Further studies are needed to confirm the possible defect in delta-4 desaturase activity and possible dietary modification of the course of this prcd retinal degeneration.
Wetzel
M G
MG
National Eye Institute, Bethesda, MD.
Fahlman
C
C
Maude
M B
MB
Alvarez
R A
RA
O'Brien
P J
PJ
Acland
G M
GM
Aguirre
G D
GD
Anderson
R E
RE
D000818:Animals; D002851:Chromatography, High Pressure Liquid; D002855:Chromatography, Thin Layer; D004195:Disease Models, Animal; D004281:Docosahexaenoic Acids / Q000097:blood / Q000378:metabolism; D004285:Dogs; D005227:Fatty Acids / Q000097:blood / Q000378:metabolism*; D008055:Lipids / Q000097:blood; D010743:Phospholipids / Q000378:metabolism; D010786:Photoreceptor Cells / Q000378:metabolism*; D012162:Retinal Degeneration / Q000378:metabolism*; D012374:Rod Cell Outer Segment / Q000378:metabolism*
D005227:Fatty Acids; D008055:Lipids; D010743:Phospholipids; D004281:Docosahexaenoic Acids