TY  - JOUR
AU  - Snijder, Pauline M.
AU  - Baratashvili, Madina
AU  - Grzeschik, Nicola A.
AU  - Leuvenink, Henri G. D.
AU  - Kuijpers, Lucas
AU  - Huitema, Sippie
AU  - Schaap, Onno
AU  - Giepmans, Ben N. G.
AU  - Kuipers, Jeroen
AU  - Miljković, Jan Lj
AU  - Mitrović, Aleksandra D.
AU  - Bos, Eelke M.
AU  - Szabo, Csaba
AU  - Kampinga, Harm H.
AU  - Dijkers, Pascale F.
AU  - den Dunnen, Wilfred F. A.
AU  - Filipović, Miloš R.
AU  - van Goor, Harry
AU  - Sibon, Ody C. M.
PY  - 2015
UR  - https://cherry.chem.bg.ac.rs/handle/123456789/2255
AB  - Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine (polyQ) disorder caused by a CAG repeat expansion in the ataxin-3 (ATXN3) gene resulting in toxic protein aggregation. Inflammation and oxidative stress are considered secondary factors contributing to the progression of this neurodegenerative disease. There is no cure that halts or reverses the progressive neurodegeneration of SCA3. Here we show that overexpression of cystathionine.-lyase, a central enzyme in cysteine metabolism, is protective in a Drosophila model for SCA3. SCA3 flies show eye degeneration, increased oxidative stress, insoluble protein aggregates, reduced levels of protein persulfidation and increased activation of the innate immune response. Overexpression of Drosophila cystathionine.-lyase restores protein persulfidation, decreases oxidative stress, dampens the immune response and improves SCA3-associated tissue degeneration. Levels of insoluble protein aggregates are not altered; therefore, the data implicate a modifying role of cystathionine.-lyase in ameliorating the downstream consequence of protein aggregation leading to protection against SCA3-induced tissue degeneration. The cystathionine.-lyase expression is decreased in affected brain tissue of SCA3 patients, suggesting that enhancers of cystathionine.-lyase expression or activity are attractive candidates for future therapies.
PB  - Feinstein Inst Med Res, Manhasset
T2  - Molecular Medicine
T1  - Overexpression of Cystathionine gamma-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3
VL  - 21
SP  - 758
EP  - 768
DO  - 10.2119/molmed.2015.00221
ER  - 

@article{
author = "Snijder, Pauline M. and Baratashvili, Madina and Grzeschik, Nicola A. and Leuvenink, Henri G. D. and Kuijpers, Lucas and Huitema, Sippie and Schaap, Onno and Giepmans, Ben N. G. and Kuipers, Jeroen and Miljković, Jan Lj and Mitrović, Aleksandra D. and Bos, Eelke M. and Szabo, Csaba and Kampinga, Harm H. and Dijkers, Pascale F. and den Dunnen, Wilfred F. A. and Filipović, Miloš R. and van Goor, Harry and Sibon, Ody C. M.",
year = "2015",
abstract = "Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine (polyQ) disorder caused by a CAG repeat expansion in the ataxin-3 (ATXN3) gene resulting in toxic protein aggregation. Inflammation and oxidative stress are considered secondary factors contributing to the progression of this neurodegenerative disease. There is no cure that halts or reverses the progressive neurodegeneration of SCA3. Here we show that overexpression of cystathionine.-lyase, a central enzyme in cysteine metabolism, is protective in a Drosophila model for SCA3. SCA3 flies show eye degeneration, increased oxidative stress, insoluble protein aggregates, reduced levels of protein persulfidation and increased activation of the innate immune response. Overexpression of Drosophila cystathionine.-lyase restores protein persulfidation, decreases oxidative stress, dampens the immune response and improves SCA3-associated tissue degeneration. Levels of insoluble protein aggregates are not altered; therefore, the data implicate a modifying role of cystathionine.-lyase in ameliorating the downstream consequence of protein aggregation leading to protection against SCA3-induced tissue degeneration. The cystathionine.-lyase expression is decreased in affected brain tissue of SCA3 patients, suggesting that enhancers of cystathionine.-lyase expression or activity are attractive candidates for future therapies.",
publisher = "Feinstein Inst Med Res, Manhasset",
journal = "Molecular Medicine",
title = "Overexpression of Cystathionine gamma-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3",
volume = "21",
pages = "758-768",
doi = "10.2119/molmed.2015.00221"
}

Snijder, P. M., Baratashvili, M., Grzeschik, N. A., Leuvenink, H. G. D., Kuijpers, L., Huitema, S., Schaap, O., Giepmans, B. N. G., Kuipers, J., Miljković, J. L., Mitrović, A. D., Bos, E. M., Szabo, C., Kampinga, H. H., Dijkers, P. F., den Dunnen, W. F. A., Filipović, M. R., van Goor, H.,& Sibon, O. C. M.. (2015). Overexpression of Cystathionine gamma-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. in Molecular Medicine
Feinstein Inst Med Res, Manhasset., 21, 758-768.
https://doi.org/10.2119/molmed.2015.00221

Snijder PM, Baratashvili M, Grzeschik NA, Leuvenink HGD, Kuijpers L, Huitema S, Schaap O, Giepmans BNG, Kuipers J, Miljković JL, Mitrović AD, Bos EM, Szabo C, Kampinga HH, Dijkers PF, den Dunnen WFA, Filipović MR, van Goor H, Sibon OCM. Overexpression of Cystathionine gamma-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. in Molecular Medicine. 2015;21:758-768.
doi:10.2119/molmed.2015.00221 .

Snijder, Pauline M., Baratashvili, Madina, Grzeschik, Nicola A., Leuvenink, Henri G. D., Kuijpers, Lucas, Huitema, Sippie, Schaap, Onno, Giepmans, Ben N. G., Kuipers, Jeroen, Miljković, Jan Lj, Mitrović, Aleksandra D., Bos, Eelke M., Szabo, Csaba, Kampinga, Harm H., Dijkers, Pascale F., den Dunnen, Wilfred F. A., Filipović, Miloš R., van Goor, Harry, Sibon, Ody C. M., "Overexpression of Cystathionine gamma-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3" in Molecular Medicine, 21 (2015):758-768,
https://doi.org/10.2119/molmed.2015.00221 . .

TY  - DATA
AU  - Snijder, Pauline M.
AU  - Baratashvili, Madina
AU  - Grzeschik, Nicola A.
AU  - Leuvenink, Henri G. D.
AU  - Kuijpers, Lucas
AU  - Huitema, Sippie
AU  - Schaap, Onno
AU  - Giepmans, Ben N. G.
AU  - Kuipers, Jeroen
AU  - Miljković, Jan Lj
AU  - Mitrović, Aleksandra D.
AU  - Bos, Eelke M.
AU  - Szabo, Csaba
AU  - Kampinga, Harm H.
AU  - Dijkers, Pascale F.
AU  - den Dunnen, Wilfred F. A.
AU  - Filipović, Miloš R.
AU  - van Goor, Harry
AU  - Sibon, Ody C. M.
PY  - 2015
UR  - https://cherry.chem.bg.ac.rs/handle/123456789/3402
PB  - Feinstein Inst Med Res, Manhasset
T2  - Molecular Medicine
T1  - Supplementary data for article: Snijder, P. M.; Baratashvili, M.; Grzeschik, N. A.; Leuvenink, H. G. D.; Kuijpers, L.; Huitema, S.; Schaap, O.; Giepmans, B. N. G.; Kuipers, J.; Miljkovic, J. L.; et al. Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. Molecular Medicine 2015, 21, 758–768. https://doi.org/10.2119/molmed.2015.00221
UR  - https://hdl.handle.net/21.15107/rcub_cherry_3402
ER  - 

@misc{
author = "Snijder, Pauline M. and Baratashvili, Madina and Grzeschik, Nicola A. and Leuvenink, Henri G. D. and Kuijpers, Lucas and Huitema, Sippie and Schaap, Onno and Giepmans, Ben N. G. and Kuipers, Jeroen and Miljković, Jan Lj and Mitrović, Aleksandra D. and Bos, Eelke M. and Szabo, Csaba and Kampinga, Harm H. and Dijkers, Pascale F. and den Dunnen, Wilfred F. A. and Filipović, Miloš R. and van Goor, Harry and Sibon, Ody C. M.",
year = "2015",
publisher = "Feinstein Inst Med Res, Manhasset",
journal = "Molecular Medicine",
title = "Supplementary data for article: Snijder, P. M.; Baratashvili, M.; Grzeschik, N. A.; Leuvenink, H. G. D.; Kuijpers, L.; Huitema, S.; Schaap, O.; Giepmans, B. N. G.; Kuipers, J.; Miljkovic, J. L.; et al. Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. Molecular Medicine 2015, 21, 758–768. https://doi.org/10.2119/molmed.2015.00221",
url = "https://hdl.handle.net/21.15107/rcub_cherry_3402"
}

Snijder, P. M., Baratashvili, M., Grzeschik, N. A., Leuvenink, H. G. D., Kuijpers, L., Huitema, S., Schaap, O., Giepmans, B. N. G., Kuipers, J., Miljković, J. L., Mitrović, A. D., Bos, E. M., Szabo, C., Kampinga, H. H., Dijkers, P. F., den Dunnen, W. F. A., Filipović, M. R., van Goor, H.,& Sibon, O. C. M.. (2015). Supplementary data for article: Snijder, P. M.; Baratashvili, M.; Grzeschik, N. A.; Leuvenink, H. G. D.; Kuijpers, L.; Huitema, S.; Schaap, O.; Giepmans, B. N. G.; Kuipers, J.; Miljkovic, J. L.; et al. Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. Molecular Medicine 2015, 21, 758–768. https://doi.org/10.2119/molmed.2015.00221. in Molecular Medicine
Feinstein Inst Med Res, Manhasset..
https://hdl.handle.net/21.15107/rcub_cherry_3402

Snijder PM, Baratashvili M, Grzeschik NA, Leuvenink HGD, Kuijpers L, Huitema S, Schaap O, Giepmans BNG, Kuipers J, Miljković JL, Mitrović AD, Bos EM, Szabo C, Kampinga HH, Dijkers PF, den Dunnen WFA, Filipović MR, van Goor H, Sibon OCM. Supplementary data for article: Snijder, P. M.; Baratashvili, M.; Grzeschik, N. A.; Leuvenink, H. G. D.; Kuijpers, L.; Huitema, S.; Schaap, O.; Giepmans, B. N. G.; Kuipers, J.; Miljkovic, J. L.; et al. Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. Molecular Medicine 2015, 21, 758–768. https://doi.org/10.2119/molmed.2015.00221. in Molecular Medicine. 2015;.
https://hdl.handle.net/21.15107/rcub_cherry_3402 .

Snijder, Pauline M., Baratashvili, Madina, Grzeschik, Nicola A., Leuvenink, Henri G. D., Kuijpers, Lucas, Huitema, Sippie, Schaap, Onno, Giepmans, Ben N. G., Kuipers, Jeroen, Miljković, Jan Lj, Mitrović, Aleksandra D., Bos, Eelke M., Szabo, Csaba, Kampinga, Harm H., Dijkers, Pascale F., den Dunnen, Wilfred F. A., Filipović, Miloš R., van Goor, Harry, Sibon, Ody C. M., "Supplementary data for article: Snijder, P. M.; Baratashvili, M.; Grzeschik, N. A.; Leuvenink, H. G. D.; Kuijpers, L.; Huitema, S.; Schaap, O.; Giepmans, B. N. G.; Kuipers, J.; Miljkovic, J. L.; et al. Overexpression of Cystathionine γ-Lyase Suppresses Detrimental Effects of Spinocerebellar Ataxia Type 3. Molecular Medicine 2015, 21, 758–768. https://doi.org/10.2119/molmed.2015.00221" in Molecular Medicine (2015),
https://hdl.handle.net/21.15107/rcub_cherry_3402 .