Ceroid lipofuscinosis in sheep. II. The major component of the lipopigment in liver, kidney, pancreas, and brain is low molecular weight protein
- PMID: 3944108
Ceroid lipofuscinosis in sheep. II. The major component of the lipopigment in liver, kidney, pancreas, and brain is low molecular weight protein
Abstract
Previous studies on the lipopigment from the livers of sheep affected with ceroid lipofuscinosis showed that the disease does not involve a defect in lipid metabolism or abnormal lipid peroxidation and that most of the lipopigment was proteinaceous. In this study, lipopigment was isolated from liver, kidney, pancreas, and brain of affected sheep without the use of proteolytic enzymes. Lipopigment from all tissues was two-thirds protein. Modified silver staining after sodium dodecyl sulfate-polyacrylamide gel electrophoresis showed a major band of Mr = 14,800, heterogeneous material between Mr = 5,000 and 9,000, and a major band of Mr = 3,500. These compounds did not stain for RNA or carbohydrate and were digested by a nuclease-free protease as expected for protein. They are not normal lysosomal proteins. Lipopigment levels of dolichol, ubiquinone, and cholesterol were consistent with the lipopigment being protein-enriched lysosome-derived cytosomes. The presence of the Mr = 3,500 proteins in whole affected tissue homogenates distinguished them from homogenates of normal tissues. It was concluded that low Mr proteins are specifically stored in ovine ceroid lipofuscinosis and that the ceroid lipofuscinoses may result from inherited defects in lysosomal protein catabolism.
Similar articles
-
Ceroid lipofuscinosis in sheep. I. Bis(monoacylglycero)phosphate, dolichol, ubiquinone, phospholipids, fatty acids, and fluorescence in liver lipopigment lipids.J Biol Chem. 1986 Feb 5;261(4):1766-72. J Biol Chem. 1986. PMID: 3944107
-
Ovine ceroid-lipofuscinosis. I: Lipopigment composition is indicative of a lysosomal proteinosis.Am J Med Genet Suppl. 1988;5:141-58. doi: 10.1002/ajmg.1320310618. Am J Med Genet Suppl. 1988. PMID: 3146313
-
Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence.J Biol Chem. 1989 Apr 5;264(10):5736-40. J Biol Chem. 1989. PMID: 2522438
-
Lipopigment in the aging brain.Am J Med Genet Suppl. 1988;5:183-91. doi: 10.1002/ajmg.1320310621. Am J Med Genet Suppl. 1988. PMID: 3146316 Review.
-
Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses.Adv Exp Med Biol. 1989;266:211-22; discussion 223. doi: 10.1007/978-1-4899-5339-1_15. Adv Exp Med Biol. 1989. PMID: 2535017 Review.
Cited by
-
Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis).Biochem J. 1991 Apr 1;275 ( Pt 1)(Pt 1):269-72. doi: 10.1042/bj2750269. Biochem J. 1991. PMID: 1826833 Free PMC article.
-
Hepatic lipofuscinosis in healthy Norwegian sheep.Acta Vet Scand. 1990;31(1):73-8. doi: 10.1186/BF03547579. Acta Vet Scand. 1990. PMID: 2399873 Free PMC article.
-
Neuron-astrocyte interactions in neurodegenerative diseases: Role of neuroinflammation.Clin Exp Neuroimmunol. 2015 Aug;6(3):245-263. doi: 10.1111/cen3.12237. Epub 2015 Aug 3. Clin Exp Neuroimmunol. 2015. PMID: 26543505 Free PMC article.
-
Peroxynitrite induces neuronal cell death in aging and age-associated disorders: A review.J Am Aging Assoc. 2001 Jan;24(1):11-8. doi: 10.1007/s11357-001-0002-8. J Am Aging Assoc. 2001. PMID: 23604871 Free PMC article.
-
Aggregation chimeras provide evidence of in vivo intercellular correction in ovine CLN6 neuronal ceroid lipofuscinosis (Batten disease).PLoS One. 2022 Apr 11;17(4):e0261544. doi: 10.1371/journal.pone.0261544. eCollection 2022. PLoS One. 2022. PMID: 35404973 Free PMC article.
Publication types
MeSH terms
Substances
Grants and funding
LinkOut - more resources
Full Text Sources
