Yeh PA, Liu YH, Chu WC, Liu JY, Sun YH. Glial expression of disease-associated poly-glutamine proteins impairs the blood-brain barrier in Drosophila. Hum Mol Genet. 2018 Jul 15;27(14):2546-2562. PMID: 29726932.
From the abstract: "Expansion of poly-glutamine (polyQ) stretches in several proteins has been linked to neurodegenerative diseases. The effects of polyQ-expanded proteins on neurons have been extensively studied, but their effects on glia remain unclear. We found that expression of distinct polyQ proteins exclusively in all glia or specifically in the blood-brain barrier (BBB) and blood-retina barrier (BRB) glia caused cell-autonomous impairment of BBB/BRB integrity ... Furthermore, we also found that BBB/BRB leakage in Drosophila is reflected in reversed waveform polarity on the basis of electroretinography (ERG) ... The polyQ-expanded protein Atxn3-84Q forms aggregates, induces BBB/BRB leakage, restricts Drosophila lifespan and reduces the level of Repo ... Expression of Repo in BBB/BRB glia can rescue BBB/BRB leakage ... Coexpression of the chaperon HSP40 and HSP70 effectively rescues the effects of Atxn3-84Q ... coexpression of wild-type Atxn3-27Q can also rescue BBB/BRB impairment, suggesting that normal polyQ protein may have a protective function."
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