Induction of Exocytosis Rescues Lysosomal GM2 Accumulation in Tay-Sachs Disease

<div>LysoTracker staining of tocopherol- (<i>δ</i>-T-) and cyclodextrin- (CD-) treated fibroblast and neuroglia derived from (a) 5-month-old <i>Hexa-/-</i> and <i>Hexa-/-Neu3-/-</i> and (b) from human TSD patient. Total LysoTracker signal was measured by ImageJ, and graphics for LysoTracker intensity per cell were represented for (c) mouse fibroblast, (d) mouse neuroglia, (e) human fibroblast, and (f) human neuroglia. Images were taken under the same light intensity differing only for filter type at 100x magnification, and the data are represented as the <span class="nowrap"><svg height="9.10327pt" id="M32" style="vertical-align:-0.3499298pt" version="1.1" viewbox="-0.0498162 -8.75334 73.751 9.10327" width="73.751pt" xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink"><g transform="matrix(.013,0,0,-0.013,0,0)"><path d="M797 0V26C739 32 732 36 732 103V296C732 394 682 449 605 449C576 449 550 437 529 423C504 407 475 389 446 366C425 418 382 449 334 449C303 449 279 437 253 421C222 403 201 385 180 371V452C135 432 85 419 41 411V388C99 379 102 374 102 310V103C102 38 93 32 27 26V0H238V26C189 32 180 38 180 103V338C210 363 250 390 289 390C351 390 377 348 377 275V103C377 37 368 32 306 26V0H520V26C465 32 456 38 456 101V296C456 314 455 326 453 338C491 369 529 390 565 390C628 390 653 345 653 274V107C653 36 642 32 583 26V0H797Z"></path></g><g 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7H431L675 555H679L683 147C683 41 680 35 598 28V0H861Z"></path></g></svg>.</span> Two-way ANOVA analysis was used to determine <svg height="10.2124pt" id="M33" style="vertical-align:-3.42943pt" version="1.1" viewbox="-0.0498162 -6.78297 7.83752 10.2124" width="7.83752pt" xmlns="http://www.w3.org/2000/svg" xmlns:xlink="http://www.w3.org/1999/xlink"><g transform="matrix(.013,0,0,-0.013,0,0)"><path d="M570 304C570 398 525 448 414 448C385 448 343 445 312 434L329 511L321 518C297 504 262 482 244 460L233 411C195 397 159 381 128 358L135 332C160 347 189 360 224 373L111 -147C97 -210 84 -218 17 -231L13 -257L254 -247L259 -218L233 -216C183 -212 177 -202 189 -142L218 -1C238 -10 266 -12 283 -12C351 3 429 48 483 105C543 168 570 242 570 304ZM482 289C482 161 380 33 304 33C278 33 248 51 233 69L303 396C326 400 352 403 369 403C428 403 482 380 482 289Z"></path></g></svg> values via GraphPad (<span class="nowrap"><svg height="13.532pt" id="M34" style="vertical-align:-3.4295pt" version="1.1" 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Advances in Cell and Gene Therapy

fig3

Figure 3

Figure 3: Induction of Exocytosis Rescues Lysosomal GM2 Accumulation in Tay-Sachs Disease 