Tissue culture loading test with storage granules from animal models of neuronal ceroid‐lipofuscinosis (Batten disease): Testing their lysosomal degradability by normal and Batten cells
- 5 June 1995
- journal article
- research article
- Published by Wiley in American Journal of Medical Genetics
- Vol. 57 (2), 213-221
- https://doi.org/10.1002/ajmg.1320570220
Abstract
Storage granules (SGs) from ovine and canine models of Batten disease were found to be easily phagocytosed by four cell types studied. The cell types tested were human fibroblasts and peripheral monocytes (control and from a late infantile Batten disease patient), rat C6 cell line, and neonatal cardiomyocytes. The phagocytosed SGs elicited an increase in acid phosphatase activity which was localized in the phagolysosome. After phagocytosis SGs were followed for various times ranging from 7 to 21 days and were found to be of unchanged density (phase contrast), autofluorescence, and ultrastructural appearance. These findings point to their undegradability, or very low degree of degradability, in phagolysosomes in both normal or Batten cultured cells. The Batten disease SGs are not toxic and did not cause any adverse affect on the host cells. Either the normal clearance rate from lysosomes is too slow to be measured by this technique or subunit c accumulation in lysosomes need not result from a primary lysosomal protease defect. Subunit c may aggregate, because of the lack of some normally preventive factor, resulting in a physical barrier to the degradation of this highly apolar molecule.Keywords
This publication has 25 references indexed in Scilit:
- Immunocytochemical studies in the ceroid-lipofuscinoses (Batten disease) using antibodies to subunit c of mitochondrial ATP synthaseAmerican Journal of Medical Genetics, 1995
- Specific Delay of Degradation of Mitochondrial ATP Synthase Subunit c in Late Infantile Neuronal Ceroid Lipofuscinosis (Batten Disease)Journal of Neurochemistry, 1995
- Storage of saposins A and D in infantile neuronal ceroid‐lipofuscinosisFEBS Letters, 1993
- Refined Assignment of the Infantile Neuronal Ceroid Lipofuscinosis (INCL, CLN1) Locus at 1p32: Incorporation of Linkage Disequilibrium in Multipoint AnalysisGenomics, 1993
- Fine Genetic Mapping of the Batten Disease Locus (CLN3) by Haplotype Analysis and Demonstration of Allelic Association with Chromosome 16p Microsatellite LociGenomics, 1993
- Erythrophagocytosis by cultured skin fibroblasts from patients with hereditary metabolic disordersCellular and Molecular Life Sciences, 1993
- Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease)American Journal of Medical Genetics, 1992
- Stored dolichyl pyrophosphoryl oligosaccharides in Batten diseaseAmerican Journal of Medical Genetics, 1992
- Ovine ceroid-lipofuscinosis I: Lipopigment composition is indicative of a lysosomal proteinosisAmerican Journal of Medical Genetics, 1988
- Amyloid Deposits and AmyloidosisNew England Journal of Medicine, 1980