期刊
JOURNAL OF NEUROCHEMISTRY
卷 143, 期 2, 页码 236-243出版社
WILEY
DOI: 10.1111/jnc.14110
关键词
frontotemporal lobar degeneration; lysosomal storage diseases; lysosome; neuronal ceroid lipofuscinosis; progranulin; prosaposin
资金
- Association of Frontotemporal Dementia (AFTD)
- NINDS [R01NS088448]
- Weill Institute
- Harry and Samuel Mann Outstanding Graduate Student Award
- NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE [R01NS088448, R21NS081357, R01NS095954] Funding Source: NIH RePORTER
The frontotemporal lobar degeneration (FTLD) protein progranulin (PGRN) is essential for proper lysosomal function. PGRN localizes in the lysosomal compartment within the cell. Prosaposin (PSAP), the precursor of lysosomal saposin activators (saposin A, B, C, D), physically interacts with PGRN. Previously, we have shown that PGRN and PSAP facilitate each other's lysosomal trafficking. Here, we report that the interaction between PSAP and PGRN requires the linker region of saposin B and C (BC linker). PSAP protein with the BC linker mutated, fails to interact with PGRN and deliver PGRN to lysosomes in the biosynthetic and endocytic pathways. On the other hand, PGRN interacts with PSAP through multiple granulin motifs. Granulin D and E bind to PSAP with similar affinity as full-length PGRN.
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