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• W2605084038 endingPage "2334" @default.
• W2605084038 startingPage "2321" @default.
• W2605084038 abstract "Mutations of various genes cause hereditary spastic paraplegia (HSP), a neurological disease involving dying-back degeneration of upper motor neurons. From these, mutations in the SPAST gene encoding the microtubule-severing protein spastin account for most HSP cases. Cumulative genetic and experimental evidence suggests that alterations in various intracellular trafficking events, including fast axonal transport (FAT), may contribute to HSP pathogenesis. However, the mechanisms linking SPAST mutations to such deficits remain largely unknown. Experiments presented here using isolated squid axoplasm reveal inhibition of FAT as a common toxic effect elicited by spastin proteins with different HSP mutations, independent of microtubule-binding or severing activity. Mutant spastin proteins produce this toxic effect only when presented as the tissue-specific M1 isoform, not when presented as the ubiquitously-expressed shorter M87 isoform. Biochemical and pharmacological experiments further indicate that the toxic effects of mutant M1 spastins on FAT involve casein kinase 2 (CK2) activation. In mammalian cells, expression of mutant M1 spastins, but not their mutant M87 counterparts, promotes abnormalities in the distribution of intracellular organelles that are correctable by pharmacological CK2 inhibition. Collectively, these results demonstrate isoform-specific toxic effects of mutant M1 spastin on FAT, and identify CK2 as a critical mediator of these effects." @default.
• W2605084038 created "2017-04-14" @default.
• W2605084038 creator A5021554501 @default.
• W2605084038 creator A5031649084 @default.
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• W2605084038 creator A5058133079 @default.
• W2605084038 creator A5062867075 @default.
• W2605084038 creator A5070073548 @default.
• W2605084038 creator A5071375111 @default.
• W2605084038 creator A5072020119 @default.
• W2605084038 creator A5090468995 @default.
• W2605084038 date "2017-04-07" @default.
• W2605084038 modified "2023-10-16" @default.
• W2605084038 title "Mutant spastin proteins promote deficits in axonal transport through an isoform-specific mechanism involving casein kinase 2 activation" @default.
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