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Proceedings of the National Academy of Sciences of the United States of America 105, 1444714452 (2008)

8.3 Other potential indications Besides bronchiectasis and COPD, DPP1 inhibitors have shown important application potential in research for various other indications

Mutations in PDSS2, COQ6 , and ADCK4/COQ8B affect mainly the kidney by inducing steroid-resistant nephrotic syndrome while COQ2 mutations induce multisystem disorders whose severity correlates with the mutated genotype (Desbats et al., COQ4, COQ7, COQ9 , and/or PDSS1 develop encephalopathy and those affected by mutations in ADCK3/COQ8A develop mainly cerebellar disorders

IL-1R1 and TLR4 are expressed in astrocytes and BBB ECs and have been shown to be upregulated in clinical and experimental studies of epilepsy