Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration.
Swetha MohanPaul J SampognaroAndrea R ArgouarchJason C MaynardMackenzie WelchAnand PatwardhanEmma C CourtneyJiasheng ZhangAmanda MasonKathy H LiEric J HuangWilliam W SeeleyBruce L MillerAlma BurlingameMathew P JacobsonAimee W KaoPublished in: Molecular neurodegeneration (2021)
This study demonstrates that multiple lysosomal proteases may work in concert to liberate multi-granulin fragments and granulins. It also implicates both AEP and granulin F in the neurobiology of FTLD-TDP-Pgrn. Modulating progranulin cleavage and granulin production may represent therapeutic strategies for FTLD-Pgrn and other progranulin-related diseases.