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Progranulin Pathway
- Molecular classification
- Secreted glycoprotein, Lysosomal protein, Growth factor
01
Overview
The progranulin pathway encompasses the synthesis, trafficking, processing, and functional roles of progranulin, a secreted glycoprotein involved in lysosomal function, neuroprotection, and inflammation regulation. Progranulin is cleaved into granulins, which may have distinct biological activities. The pathway involves interactions with proteins such as sortilin, prosaposin, Surf4, and TMEM106B. Deficiency in functional progranulin leads to frontotemporal dementia and neuronal ceroid lipofuscinosis, highlighting its essential role in neuronal health and proper functioning of cellular degradation pathways.
02
Biological functions
Lysosomal regulationNeuroprotectionInflammation regulationProtein processing
03
Disease associations
Frontotemporal dementiaNeuronal ceroid lipofuscinosis
04
Safety considerations
Potential for off-target effects due to broad expression profileComplexity of granulins' distinct functions requiring careful modulation
05
Biomarkers
Progranulin levels (CSF/plasma) as diagnostic/prognostic marker in FTD
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