Výsledky vyhledávání - "W. Pijnappel"

Modelling the neuropathology of lysosomal storage disorders through disease-specific human induced pluripotent stem cells

Autor: Bálint Jezsó, Phetcharat Phanthong, Kinga Molnár, Poul Hyttel, Maria Lo Giudice, Marianne Hoogeveen-Westerveld, Lajos László, W. Pijnappel, Shuling Zhou, Eszter Varga, Kristine K. Freude, István Bock, Annamária Téglási, Hideyuki Nakanishi, Norbert Varga, Andras Dinnyes, Narisorn Kitiyanant, Julianna Kobolák

Publikováno v: Experimental Cell Research, 380(2), 216-233. Elsevier Inc.
Kobolák, J, Molnár, K, Varga, E, Bock, I, Jezsó, B, Téglási, A, Zhou, S, Lo Giudice, M, Hoogeveen-Westerveld, M, Pijnappel, WWM P, Phanthong, P, Varga, N, Kitiyanant, N, Freude, K, Nakanishi, H, László, L, Hyttel, P & Dinnyés, A 2019, ' Modelling the neuropathology of lysosomal storage disorders through disease-specific human induced pluripotent stem cells ', Experimental Cell Research, vol. 380, no. 2, pp. 216-233 . https://doi.org/10.1016/j.yexcr.2019.04.021
Experimental Cell Research

Mucopolysaccharidosis II (MPS II) is a lysosomal storage disorder (LSD), caused by iduronate 2-sulphatase (IDS) enzyme dysfunction. The neuropathology of the disease is not well understood, although the neural symptoms are currently incurable. MPS II

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https://doi.org/10.1016/j.yexcr.2019.04.021

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Additional file 2: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S2. Modest muscle regeneration in Gaa−/−(Bl6) animals. A.eMyHC staining of QF sections from WT(Bl6) and Gaa−/−(Bl6) animals. The figure shows selected areas of eMyHC (red)/laminin(green)/Hoechst(blue) stained QF sections from 4, 12 and

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Additional file 7: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S7. Detailed histological evaluation of regenerating WT and Gaa−/− muscle. The figure depicts HE- and trichrome stained histological sections from 15 week WT(FVB) and Gaa−/− (FVB) TA muscles at multiple time-points during the first 15

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Additional file 5: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S5. Satellite cell numbers are increased in Gaa−/−(Bl6) muscle. A. Satellite cells were detected in 3 months old WT(Bl6) and Gaa−/−(Bl6) gastrocnemius (GAS) cryosections by immunofluorescent staining of Pax7 (red). Myofibers were visua

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Additional file 8: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S8. Gaa−/− muscle regenerates completely without tissue remodeling Depicted are images from trichrome staining of TA muscle of WT(FVB) and Gaa−/−(FVB) at 10, 25 and 40 weeks before and 15 days after BaCl2 injury. (PDF 348 kb)

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Additional file 9: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S9. Efficient regeneration of GAAKO-muscle after cardiotoxin-induced injury in GAA-deficient animals on a C57/Bl6 background. The figure shows histological sections from TA HE-stained sections from 12 and 48 week old WT(Bl6) and Gaa−/− (Bl

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Additional file 3: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S3. Satellite cell numbers are increased in Gaa−/− TA muscle. The number of Pax7-positive cells in 15 week (A) and 25 week (B) WT and Gaa−/− from Fig. 3 expressed as Pax7-positive cells/myofiber. Data are means ± SD from 2 muscles der

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Additional file 1: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Stijn In ‘T Groen, Jong, Bart De, Boomaars, Björn, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S1. Reduced fiber diameter in GAAKO animals. A. Fiber diameter frequency distribution plot of WT(FVB) and Gaa−/−(FVB) at 15 weeks of age. B. WT(Bl6) and GAAKO(Bl6) at 3 months of age showing reduced fiber diameter in Gaa−/− (Bl6). Thes

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Additional file 6: of Satellite cells maintain regenerative capacity but fail to repair disease-associated muscle damage in mice with Pompe disease

Autor: Schaaf, Gerben, Gestel, Tom Van, Groen, Stijn In, Jong, Bart De, BjĂśrn Boomaars, Tarallo, Antonietta, Cardone, Monica, Parenti, Giancarlo, Ploeg, Ans Van Der, W. Pijnappel

Figure S6. Detection of proliferating satellite cells in GAA-deficient limb muscle. Representative images from TA limb muscle sections co-stained for Pax7 (red) and Ki67 (green) to detect proliferating satellite cells (arrow). Nuclei are visualized w

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