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Author

Martin S. King

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Open access Sep 2026

The Batten disease protein CLN8 is a stereospecific acyltransferase in bis(monoacylglycero)phosphate biosynthesis

Loss-of-function mutations in the endoplasmic reticulum membrane protein CLN8 cause Batten disease, a neurodegenerative lysosomal storage disorder. CLN8 acts with the lysosomal enzyme CLN5 to produce bis(monoacylglycero)phosphate (BMP), a signature lysosomal phospholipid with unique S,S stereochemistry. However, the ro...

P. K. Sheokand, Denis Lacabanne, A. James et al. · 3 citations · ⚡1
Open access Sep 2026

Tissue-wide metabolic buffering confers resilience to mitochondrial dysfunction

Mitochondria and oxidative phosphorylation (OxPhos) are essential for cellular homeostasis. However, the phenotypes caused by mitochondrial dysfunction often display remarkable tissue-specificity. What determines the susceptibility of individual cells to metabolic or mitochondrial defects in a complex in vivo tissue co...

S. Petridi, Abhilesh Dhawanjewar, Dnyanesh Dubal et al. · 0 citations

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