Impaired cholesterol esterification in primary brain cultures of the lysosomal cholesterol storage disorder (LCSD) mouse mutant
| Autor: | Shutish Patel, Sundar Suresh, Peter G. Pentchev, Hana Weintroub, Roscoe O. Brady |
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| Rok vydání: | 1987 |
| Předmět: |
Male
medicine.medical_specialty Mutant Central nervous system Biophysics Oleic Acids Biology Tritium Biochemistry Lipid Metabolism Inborn Errors Mice chemistry.chemical_compound Internal medicine medicine Organoid Animals Molecular Biology Cells Cultured Cholesterol Homozygote Brain Heterozygote advantage Cell Biology Phenotype Mice Mutant Strains Endocrinology medicine.anatomical_structure chemistry Cell culture Mutation Female lipids (amino acids peptides and proteins) Cholesterol Esters Lysosomes Cholesterol storage Triolein Oleic Acid |
| Zdroj: | Biochemical and Biophysical Research Communications. 143:233-240 |
| ISSN: | 0006-291X |
| Popis: | Esterification of cholesterol was investigated in primary neuroglial cultures obtained from newborn lysosomal cholesterol storage disorder (LCSD) mouse mutants. An impairment in 3H-oleic acid incorporation into cholesteryl esters was demonstrated in cultures of homozygous LCSD brain. Primary cultures derived from other phenotypically normal pups of the carrier breeders esterified cholesterol at normal levels or at levels which were intermediary between normal and deficient indicating a phenotypic expression of the LCSD heterozygote genotype. These observations on LCSD mutant brain cells indicate that the defect in cholesterol esterification is closely related to the primary genetic defect and is expressed in neuroglial cells in culture. |
| Databáze: | OpenAIRE |
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