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The Batten disease gene product CLN5 is the lysosomal bis(monoacylglycero)phosphate synthase.
Science. 2023 Sep 15;381(6663):1182-1189. doi: 10.1126/science.adg9288. Epub 2023 Sep 14.
Science. 2023.
PMID: 37708259
CLN3 is required for the clearance of glycerophosphodiesters from lysosomes.
Laqtom NN, Dong W, Medoh UN, Cangelosi AL, Dharamdasani V, Chan SH, Kunchok T, Lewis CA, Heinze I, Tang R, Grimm C, Dang Do AN, Porter FD, Ori A, Sabatini DM, Abu-Remaileh M.
Laqtom NN, et al. Among authors: medoh un.
Nature. 2022 Sep;609(7929):1005-1011. doi: 10.1038/s41586-022-05221-y. Epub 2022 Sep 21.
Nature. 2022.
PMID: 36131016
Free PMC article.
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Glycerophosphodiesters inhibit lysosomal phospholipid catabolism in Batten disease.
Nyame K, Hims A, Aburous A, Laqtom NN, Dong W, Medoh UN, Heiby JC, Xiong J, Ori A, Abu-Remaileh M.
Nyame K, et al. Among authors: medoh un.
Mol Cell. 2024 Apr 4;84(7):1354-1364.e9. doi: 10.1016/j.molcel.2024.02.006. Epub 2024 Mar 5.
Mol Cell. 2024.
PMID: 38447580
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The Bis(monoacylglycero)-phosphate Hypothesis: From Lysosomal Function to Therapeutic Avenues.
Medoh UN, Abu-Remaileh M.
Medoh UN, et al.
Annu Rev Biochem. 2024 Apr 11. doi: 10.1146/annurev-biochem-092823-113814. Online ahead of print.
Annu Rev Biochem. 2024.
PMID: 38603559
Review.
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