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Table 2 Model systems used to study UPS function in polyglutamine expansion disorders. See text for further details.

From: The ubiquitin proteasome system in Huntington's disease and the spinocerebellar ataxias

Model Methods used in conjunction Additional notes Reference
Human post mortem brain Immunocytochemistry, assay of proteasome activity in lysates using fluorescent substrates   [3, 20, 30]
Human patient skin fibroblasts Proteasome activity using fluorescent substrates   [30]
R6/2 transgenic mouse model of HD Proteasome activity, immunocytochemistry, R6/2 mouse generated by Mangiarini et al. [60] [21, 34]
R6/1 transgenic mouse model of HD Proteasome activity R6/1 mouse generated by Mangiarini et al. [60] [15]
HD94 conditional mouse model of HD Chymotrypsin-like, trypsin-like or peptidyl-glutamyl activity in lysates HD94 conditional mouse generated by Yamamoto et al. [61] [33]
SCA 7 knock-in mouse model Crossed to a transgenic mouse expressing an EGFP–degron reporter SCA 7 transgenic mouse generated by Yoo et al. [62] [31]
Transgenic Drosophila models of polyglutamine expansion disorders Genetic screens   [56]
Cell models of HD and SCA, stable cell lines and transient expression of mutant constructs. Inducible or constitutive transgene expression Immunocytochemistry, assay of proteasome activity in lysates using fluorescent substrates, co-expression of construct with fluorescent–degron reporter.   [13-15, 20, 29, 32]
C. elegans models Co-expression of mutant ataxin-3 and a ubiquitin-conjugated dsRed reporter   [37]