Lysosomal catabolism depends on bis(monoacylglycero)phosphate (BMP), a phospholipid with atypical S,S stereochemistry of unknown biosynthetic origin. CLN8 and CLN5 catalyze sequential acylation steps in BMP synthesis, and their loss of function causes Batten disease, a group of inherited neurodegenerative lysosomal sto...
P. K. Sheokand, A. James, Christian Posern et al.· bioRxiv· 0 citations
Two studies show that the ER-associated protein CLN8 catalyses the acyl CoA-dependent acylation of glycerophosphoglycerol, producing lysophosphatidylglycerol, which is then converted into BMP, providing insight into CLN8 Batten disease.
Johannes Breithofer, Nermeen Fawzy, C. Zitta et al.· Nature Cell Biology· 3 citations
The integration of multi-omics data revealed the activation of compensatory mechanisms aimed at restoring cellular homeostasis, including metabolic rewiring and stress-response pathways, although these responses were insufficient to counteract nanosystem-induced cytotoxicity.
M. P. Buendia-Nacarino, Dominik Bulfon, Nikola Tom et al.· Pharmaceutics· 1 citation
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