Drosophila models uncover substrate channeling effects on phospholipids and sphingolipids in peroxisomal biogenesis disorders - Wangler, Michael F.; Chao, Yu-Hsin; Roth, Mary; Welti, Ruth; McNew, James A. (PloS one, 2025)
Keywords: Abnormalities; Animals; ATPases Associated with Diverse Cellular Activities; Biochemistry; Biology and Life Sciences; Biomarkers; Biosynthesis; Blood plasma; Causes of; Cell membranes; Ceramide; Channeling; Children; Cloning; Defects; Diagnosis; Diglycerides; Disease Models, Animal; Diseases; Disorders; Drosophila; Drosophila melanogaster - genetics; Drosophila melanogaster - metabolism; Drosophila Proteins - genetics; Drosophila Proteins - metabolism; Evolution; Experiments; Fatty acids; Fruit flies; Gas flow; Genes; Health aspects; Humans; Infants (Newborn); Insects; Larvae; Lecithin; Lipid metabolism; Lipids; Medical screening; Medicine and Health Sciences; Membrane Proteins - genetics; Metabolism; Metabolites; Metabolomics; Mutation; Nervous system; Peroxins - genetics; Peroxisomal disorders; Peroxisomal Disorders - genetics; Peroxisomal Disorders - metabolism; Peroxisomal Targeting Signal 2 Receptor - genetics; Peroxisomes - metabolism; Phosphatidylcholine; Phosphatidylethanolamine; Phospholipids; Phospholipids - metabolism; Physiological aspects; Plasma; Research and Analysis Methods; Sphingolipids; Sphingolipids - metabolism; Sphingomyelin; Zellweger Syndrome - genetics; Zellweger Syndrome - metabolism; United States; United States--US