Spreadsheet: 9008b82d-a108-49bb-9237-88d80030805e
Sheet: Sheet1

Table S2. Human genes encoding YFP-ATXN1Q82NT toxicity or aggregation modulators. | Unnamed: 1 | Unnamed: 2 | Unnamed: 3 | Unnamed: 4 | Unnamed: 5 | Unnamed: 6 | Unnamed: 7 | Unnamed: 8 | Unnamed: 9 | Unnamed: 10 | Unnamed: 11 | Unnamed: 12 | Unnamed: 13 | Unnamed: 14 | Unnamed: 15 | Unnamed: 16 | Unnamed: 17 | Unnamed: 18 | Unnamed: 19
Protein name | [NAME] | Expected protein MW (kDa) | In-gel protein MW (kDa) | [NAME] toxicity | ATXN1Q82NT toxicity change % | STDEV | p value | ATXN3Q73 toxicity | ATXN3Q73 toxicity change % | STDEV | p value | ATXN1Q82NT aggregation | ATXN1Q82NT aggregation change % | STDEV | p value | ATXN3Q73 aggregation | ATXN3Q73 aggregation change % | STDEV | p value
aSNAP | NAPA | 34.2 | 37 | E | 101.5 | 54.44722215136417 | 0.0840075137855968 | NC |  |  |  | E | 41.5 | 16.26345596729059 | 0.0475695654717689 | NC
L10 | RPL10 | 25.6 | 29 | E | 98.66666666666667 | 22.0302821891444 | 0.01621488291636886 | NC |  |  |  | E | 38.00000000000001 | 18.52025917745213 | 0.07086483853085848 | NC
Lin-10 | C16orf70 | 48.5 | 51 | E | 60.33333333333334 | 26.15976554431122 | 0.05733002322078486 | NC |  |  |  | E | 41 | 16.97056274847714 | 0.05264016513824565 | NC
Ran | RAN | 25.4 | 27 | E | 50.5 | 34.64823227814083 | 0.1275688447184027 | E | 25 | 2.82842712474619 | 0.05082134732351747 | E | 69 | 29.20616373302047 | 0.05485363837655979 | E | 190 | 45.7 | 0.01874382041289979
ATP5F1 | ATP5F1 | 29.9 | 32 | E | 49.66666666666666 | 11.06044001535803 | 0.01613186255260415 | NC |  |  |  | E | 77 | 32.52691193458121 | 0.05465119178172543 | NC
P0 | RPLP0 | 34.3 | 40 | E | 46.5 | 27.57716446627536 | 0.0999672198383314 | E | 29 | 2.82842712474619 | 0.04383540253734537 | NC |  |  |  | NC
Vps4B | VPS4B | 50.3 | 55 | E | 38.00000000000001 | 15.52417469626001 | 0.0513823934104619 | E | 26 | 4.242640687119285 | 0.0731327183783266 | NC |  |  |  | E | 87 | 58 | 0.1216899342953892
L8 | [NAME] | 29 | 33 | E | 34.66666666666667 | 17.67295485574875 | 0.07678568842112467 | NC |  |  |  | E | 62.33333333333334 | 23.2880512996114 | 0.04351267053642056 | NC
[NAME] | [NAME] | 38 | 45 | E | 31 | 10.14889156509225 | 0.03391938670555177 | NC |  |  |  | E | 44.66666666666666 | 9.60902353693307 | 0.0150785654127119 | NC
ATP6V1E | ATP6V1E1 | 27.2 | 28 | E | 30 | 0 | 0.0003701647359517998 | NC |  |  |  | E | 46.5 | 33.23401871576773 | 0.1363056917582549 | NC
Sm-D2 | SNRPD2 | 14.5 | 16 | E | 25 | 9.539392014169454 | 0.04526357906068726 | NC |  |  |  | NC |  |  |  | NC
MED15 | MED15 | 83.6 | 87 | E | 20 | 4.242640687119289 | 0.01467072183441291 | NC |  |  |  | E | 31.5 | 2.121320343559657 | 0.001508296484284747 | NC
EIF2G | EIF2S3 | 52.1 | 55 | S | -21 | 8.398484809835 | 0.04939709837092512 | NC |  |  |  | NC |  |  |  | NC
SmB | SNRPB | 25.6 | 26 | S | -21 | 1.414213562373094 | 0.001508296484284717 | NC |  |  |  | S | -32 | 9.899494936611667 | 0.03045140410076447 | NC
DNAJB4 | DNAJB4 | 38.8 | 39 | S | -23 | 1.414213562373094 | 0.001257862132975067 | NC |  |  |  | E | 67 | 26.87005768508883 | 0.04965335699191061 | NC
PSMB6 | PSMB6 | 26.4 | 26 | S | -23.5 | 0.7071067811865462 | 0.0003016591320256317 | NC |  |  |  | S | -35 | 7.071067811865498 | 0.01333392995545188 | NC
RPSA | RPSA | 33.8 | 40 | S | -24 | 11.31370849898476 | 0.06674347473660204 | NC |  |  |  | NC |  |  |  | NC
Crk | CRK | 34.8 | 40 | S | -24.33333333333333 | 8.962886439832502 | 0.04237070670417786 | NC |  |  |  | E | 53.33333333333334 | 8.0059517483933 | 0.007427582973553795 | NC
AFG3L2 | AFG3L2 | 89.6 | 90 | S | -38.33333333333333 | 4.509249752822923 | 0.004580807324156795 | NC |  |  |  | E | 57.49999999999999 | 45.9619407771256 | 0.1625744900067607 | NC
Pum1 | PUM1 | 127.7 | 130 | S | -55.00000000000001 | 1.000000000000001 | 0.0001101746271081077 | NC |  |  |  | NC |  |  |  | NC
Nek8 | NEK8 | 75.8 | 80 | S | -55.6666666666667 | 6.7857485933617 | 0.004916677197773963 | S | -31 | 12.28820572744451 | 0.04859023877189692 | E | 29 | 7.0277706369604 | 0.01901904708553494 | NC
S=Suppression, E=Enhancement, NC=No change
Note. 202 candidate genes were tested whether their overexpression modulates [NAME]-induced cytotoxicity and/or protein aggregation in cell-based assays.
21 proteins were identified which modulated ATXN1Q82NT-induced cytotoxicity (rows D-G / yellow color). The specificity of modifiers on ATXN1Q82NT cytotoxicity
was further tested in an ATXN3Q73 cell model (rows H-K / green color). The protein products of RAN, RPLP0, ATP5F1 and NEK8 genes modulated ATXN3Q73-mediated
cytotoxicity in a similar manner. In addition, the effect of cytotoxicity modifiers on ATXN1Q82NT protein aggregation was studied using the filter retardation assay (lit) (rows L-O / red color).
15 out of 21 modifiers (71%) also affected ATXN1Q82NT protein aggregation. For each modifier the following information is given in the Table: A) the protein name; B) the gene symbol; C) the expected molecular
weight (MW) of the relevant protein; D) the in-gel MW of the modifier protein detected by western blotting; E) the effect of the modifier protein on [NAME]-induced cytotoxicity;
F) the % change in cytotoxicity levels; G) the STDEV of three independent experiments; H) the p value of ATXN1Q82NT-induced cytotoxicity change, calculated using a two-tailed Student’s t-test.
Similar information is given for the effect of modifier genes on ATXN3Q73-induced cytotoxicity and [NAME] or ATXN3Q73 protein aggregation.