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A sequential morphological study of ovine ceroid-lipofuscinosis showed that brains of affected lambs were normal at birth, grew until four months of age but then atrophied. Laminar necrosis of cerebral cortex was noted at 10 weeks, occurring first in the parietal area, then spreading to involve frontal and occipital areas while the temporal lobe was least and last affected. With progression of the disease, the laminar pattern was lost. Neuronal necrosis was accompanied by a severe astrocytosis. The granular and multilamellar storage cytosomes increased in size with age. Their structure was interpreted as paracrystalline in which repeating molecules of the dominantly stored lipid binding subunit of mitochondrial ATP synthase interact with neutral lipids and phospholipids. Abnormal cytosomes in neurons of lamb fetuses and a neonate were interpreted as early lesions which contained whorls or stacks of bilayered membrane as well as the more complex multilamellar material. The underlying anomaly leading to the storage of the lipid binding subunit of mitochondrial ATPsynthase remains to be defined. However, it is noted that this disease should be regarded as a lipid binding protein proteinosis or alternatively as a proteolipid proteinosis.

The importance of dietary fat components, such as fatty acids, in the expression of multiple genes is clear. In the case of beef cattle, fat in the form of fatty acids (saturated or unsaturated), vitamin A (mainly retinoic acid), or carotenoids (beta-carotene and lutein) is obtained from dietary feed or pasture. The aim of this work was to study the effect of fatty acids (phytanic and pristanic acids), vitamin A (all-trans and 9-cis retinoic acid), and carotenoids (beta-carotene and lutein) on the expression of PPARgamma and its coactivator PGC-1alpha during differentiation of bovine white adipose tissue. Samples were collected at slaughter from subcutaneous adipose tissue and processed in a solution containing type II collagenase for 2 h at 37 degrees C. Cells were resuspended in basal medium, Dulbecco's modified Eagle's medium containing 5% fetal bovine serum, plated on 24-well culture plates at a density of 1 x 10(4) cells/cm(2), and incubated at 37 degrees C in a 5% CO(2) atmosphere. Preadipocyte differentiation after reaching confluence was induced by various treatments: rosiglitazone (20 microM); unsaturated fatty acids: phytanic acid (25, 50, 100 microM) and pristanic acid (25, 50, 100 microM); retinoids: 9-cis retinoic acid (0.5, 0.75, 1 microM) and all-trans retinoic acid (0.5, 0.75, 1 microM); and carotenoids: beta-carotene (10, 20, 30 microM) and lutein (10, 20, 30 microM). Expression of PPARgamma and PGC-1alpha was measured in differentiated cells. Phytanic acid, all-trans retinoic acid, and 9-cis retinoic acid were the best activators of PPARgamma expression, and the combination of 9-cis and all-trans retinoic acid was the best activator of PGC-1alpha expression (P < 0.05). Therefore, these are powerful agents for the promotion of bovine adipogenesis and constitute promising compounds to be used in bovine fattening.

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