{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T19:57:38Z","timestamp":1781639858837,"version":"3.54.5"},"reference-count":38,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:00:00Z","timestamp":1773014400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:00:00Z","timestamp":1773014400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Izmir Institute of Technology"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neuromol Med"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Tay-Sachs disease is a severe neurodegenerative disorder caused by mutations in the\n                    <jats:italic>HEXA<\/jats:italic>\n                    gene, which encodes the \u03b1-subunit of the \u03b2-hexosaminidase A (HexA) enzyme. HexA deficiency leads to abnormal GM2 accumulation, eventually causing cell death and neurodegeneration. A double-knockout mouse model lacking both\n                    <jats:italic>Hexa<\/jats:italic>\n                    and\n                    <jats:italic>Neu3<\/jats:italic>\n                    genes (\n                    <jats:italic>Hexa-\/-Neu3-\/-<\/jats:italic>\n                    ,\n                    <jats:italic>DKO<\/jats:italic>\n                    ) exhibits neuropathological and clinical features similar to those of the disease, including neuroinflammation. B4Galnt1 (\u00df-1,4-N-acetyl-galactosaminyltransferase 1) is involved in lipid biosynthesis in mice. We hypothesized that creating a triple knockout model (\n                    <jats:italic>Hexa-\/-Neu3-\/-B4Galnt1-\/-<\/jats:italic>\n                    ,\n                    <jats:italic>TKO<\/jats:italic>\n                    ) could prevent excessive GM2 ganglioside accumulation and reduce disease symptoms. Molecular biology and immunohistochemistry analyses showed that GM2 ganglioside accumulation was halted in\n                    <jats:italic>TKO<\/jats:italic>\n                    mice. Preventing GM2 ganglioside accumulation alleviated neuroinflammation and neuronal death, extending lifespan by more than 18 months. Our findings suggest that knocking out\n                    <jats:italic>B4Galnt1<\/jats:italic>\n                    to block GM2 ganglioside accumulation may reverse disease symptoms in the\n                    <jats:italic>DKO<\/jats:italic>\n                    mouse model, indicating a promising, safe target for substrate-reduction therapy via siRNA silencing.\n                  <\/jats:p>","DOI":"10.1007\/s12017-026-08916-x","type":"journal-article","created":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T19:28:38Z","timestamp":1773084518000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["B4Galnt1 Deficiency Reverses Severe Neurological Symptoms in a Mouse Model of Tay-Sachs Disease"],"prefix":"10.1007","volume":"28","author":[{"given":"Selman","family":"Yanbul","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tufan Utku","family":"Cal\u0131skan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mustafa Can","family":"Turali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0243-5011","authenticated-orcid":false,"given":"Volkan","family":"Seyrantepe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,9]]},"reference":[{"issue":"17","key":"8916_CR1","doi-asserted-by":"publisher","first-page":"3615","DOI":"10.3390\/molecules30173615","volume":"30","author":"I Abidi","year":"2025","unstructured":"Abidi, I., Kocev, A. N., Babulic, J. L., Capicciotti, C. J., Walia, J., & Brockhausen, I. (2025). Characterization of human recombinant \u03b21,4-galnac-transferase B4GALNT1 and inhibition by selected compounds. Molecules, 30(17), 3615. https:\/\/doi.org\/10.3390\/molecules30173615","journal-title":"Molecules"},{"issue":"9","key":"8916_CR2","doi-asserted-by":"publisher","first-page":"2590","DOI":"10.1002\/ajmg.a.62880","volume":"188","author":"JE Alecu","year":"2022","unstructured":"Alecu, J. E., Ohmi, Y., Bhuiyan, R. H., Inamori, K., Nitta, T., Saffari, A., Jumo, H., Ziegler, M., de Gusmao, C. M., Sharma, N., Ohno, S., Manabe, N., Yamaguchi, Y., Kambe, M., Furukawa, K., Sahin, M., Inokuchi, J., Furakawa, K., & Ebrahimi-Fakhari, D. (2022). Functional validation of novel variants in b4galnt1 associated with early\u2010onset complex hereditary spastic paraplegia with impaired ganglioside synthesis. American Journal of Medical Genetics Part A, 188(9), 2590\u20132598. https:\/\/doi.org\/10.1002\/ajmg.a.62880","journal-title":"American Journal of Medical Genetics Part A"},{"key":"8916_CR3","doi-asserted-by":"publisher","first-page":"115114","DOI":"10.1016\/j.expneurol.2024.115114","volume":"385","author":"Y An","year":"2025","unstructured":"An, Y., Yao, Y., Liu, H., Xi, Y., Pi, M., Xu, R., Huang, Y., Li, S., & Gu, X. (2025). The role of the CCL5-CCR5 axis in microglial activation leading to postoperative cognitive dysfunction. Experimental Neurology, 385, 115114. https:\/\/doi.org\/10.1016\/j.expneurol.2024.115114","journal-title":"Experimental Neurology"},{"key":"8916_CR4","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1016\/j.cytogfr.2025.05.003","volume":"84","author":"AA Bufi","year":"2025","unstructured":"Bufi, A. A., Di Stefano, J., Papait, A., Silini, A. R., Parolini, O., & Ponsaerts, P. (2025). The central role of CXCL10-CXCR3 signaling in neuroinflammation and neuropathology. Cytokine & Growth Factor Reviews, 84, 20\u201334. https:\/\/doi.org\/10.1016\/j.cytogfr.2025.05.003","journal-title":"Cytokine & Growth Factor Reviews"},{"issue":"5","key":"8916_CR5","doi-asserted-by":"publisher","first-page":"2195","DOI":"10.3390\/ijms26052195","volume":"26","author":"G Canini","year":"2025","unstructured":"Canini, G., Mazzinelli, E., Nocca, G., Lattanzi, W., & Arcovito, A. (2025). Targeting glucosylceramide synthase: Innovative Drug repurposing strategies for lysosomal diseases. International Journal of Molecular Sciences, 26(5), 2195. https:\/\/doi.org\/10.3390\/ijms26052195","journal-title":"International Journal of Molecular Sciences"},{"key":"8916_CR6","doi-asserted-by":"publisher","unstructured":"Cherry, J. D., Meng, G., Daley, S., Xia, W., Svirsky, S., Alvarez, V. E., Nicks, R., Pothast, M., Kelley, H., Huber, B., Tripodis, Y., Alosco, M. L., Mez, J., McKee, A. C., & Stein, T. D. (2020). CCL2 is associated with microglia and macrophage recruitment in chronic traumatic encephalopathy. Journal of Neuroinflammation, 17(1). https:\/\/doi.org\/10.1186\/s12974-020-02036-4","DOI":"10.1186\/s12974-020-02036-4"},{"issue":"2","key":"8916_CR7","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1006\/exnr.2000.7504","volume":"166","author":"S Chiavegatto","year":"2000","unstructured":"Chiavegatto, S., Sun, J., Nelson, R. J., & Schnaar, R. L. (2000). A functional role for complex gangliosides: Motor deficits in GM2\/GD2 synthase knockout mice. Experimental Neurology, 166(2), 227\u2013234. https:\/\/doi.org\/10.1006\/exnr.2000.7504","journal-title":"Experimental Neurology"},{"issue":"7","key":"8916_CR8","doi-asserted-by":"publisher","first-page":"1065","DOI":"10.3390\/ijms17071065","volume":"17","author":"M Coutinho","year":"2016","unstructured":"Coutinho, M., Santos, J., & Alves, S. (2016). Less is more: Substrate reduction therapy for lysosomal storage disorders. International Journal of Molecular Sciences, 17(7), 1065. https:\/\/doi.org\/10.3390\/ijms17071065","journal-title":"International Journal of Molecular Sciences"},{"key":"8916_CR9","doi-asserted-by":"publisher","unstructured":"Demir, S. A., Timur, Z. K., Ate\u015f, N., Mart\u00ednez, L. A., & Seyrantepe, V. (2020). GM2 ganglioside accumulation causes neuroinflammation and behavioral alterations in a mouse model of early onset Tay-Sachs disease. Journal of Neuroinflammation, 17(1). https:\/\/doi.org\/10.1186\/s12974-020-01947-6","DOI":"10.1186\/s12974-020-01947-6"},{"issue":"5","key":"8916_CR10","doi-asserted-by":"publisher","first-page":"1082","DOI":"10.1111\/j.1476-5381.2009.00565.x","volume":"159","author":"A Friso","year":"2010","unstructured":"Friso, A., Tomanin, R., Salvalaio, M., & Scarpa, M. (2010). Genistein reduces glycosaminoglycan levels in a mouse model of mucopolysaccharidosis type II. British Journal of Pharmacology, 159(5), 1082\u20131091. https:\/\/doi.org\/10.1111\/j.1476-5381.2009.00565.x","journal-title":"British Journal of Pharmacology"},{"issue":"7","key":"8916_CR11","doi-asserted-by":"publisher","first-page":"98","DOI":"10.3390\/neurolint17070098","volume":"17","author":"M Gonz\u00e1lez-S\u00e1nchez","year":"2025","unstructured":"Gonz\u00e1lez-S\u00e1nchez, M., Ram\u00edrez-Exp\u00f3sito, M. J., & Mart\u00ednez-Martos, J. M. (2025). Advances in diagnosis, pathological mechanisms, clinical impact, and future therapeutic perspectives in Tay\u2013Sachs disease. Neurology International, 17(7), 98. https:\/\/doi.org\/10.3390\/neurolint17070098","journal-title":"Neurology International"},{"issue":"5","key":"8916_CR12","doi-asserted-by":"publisher","first-page":"403","DOI":"10.1177\/0883073820980904","volume":"36","author":"K Goodspeed","year":"2021","unstructured":"Goodspeed, K., Feng, C., Laine, M., & Lund, T. C. (2021). Aspartylglucosaminuria: Clinical presentation and potential therapies. Journal of Child Neurology, 36(5), 403\u2013414. https:\/\/doi.org\/10.1177\/0883073820980904","journal-title":"Journal of Child Neurology"},{"key":"8916_CR13","doi-asserted-by":"publisher","unstructured":"Inamori, K., Nakamura, K., Shishido, F., Hsu, J. C., Nagafuku, M., Nitta, T., Ikeda, J., Yoshimura, H., Kodaira, M., Tsuchida, N., Matsumoto, N., Uemura, S., Ohno, S., Manabe, N., Yamaguchi, Y., Togayachi, A., Aoki-Kinoshita, K. F., Nishihara, S., Furukawa, J., & Inokuchi, J. (2024). Functional evaluation of novel variants of B4GALNT1 in a patient with hereditary spastic paraplegia and the general population. Frontiers in Neuroscience, 18. https:\/\/doi.org\/10.3389\/fnins.2024.1437668","DOI":"10.3389\/fnins.2024.1437668"},{"key":"8916_CR14","doi-asserted-by":"publisher","unstructured":"Inci, O. K., & Seyrantepe, V. (2025). Combined treatment of ketogenic diet and propagermanium reduces neuroinflammation in Tay-Sachs disease mouse model. Metabolic Brain Disease, 40(3). https:\/\/doi.org\/10.1007\/s11011-025-01553-6","DOI":"10.1007\/s11011-025-01553-6"},{"issue":"4","key":"8916_CR15","doi-asserted-by":"publisher","first-page":"974","DOI":"10.1093\/brain\/awg089","volume":"126","author":"M Jeyakumar","year":"2003","unstructured":"Jeyakumar, M., Thomas, R., Elliot-Smith, E., Smith, D. A., van der Spoel, A. C., d\u2019Azzo, A., Perry, V. H., Butters, T. D., Dwek, R. A., & Platt, F. M. (2003). Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis. Brain, 126(4), 974\u2013987. https:\/\/doi.org\/10.1093\/brain\/awg089","journal-title":"Brain"},{"key":"8916_CR16","doi-asserted-by":"publisher","unstructured":"Joly-Amado, A., Hunter, J., Quadri, Z., Zamudio, F., Rocha-Rangel, P. V., Chan, D., Kesarwani, A., Nash, K., Lee, D. C., Morgan, D., Gordon, M. N., & Selenica, M. L. B. (2020). CCL2 overexpression in the brain promotes glial activation and accelerates tau pathology in a mouse model of tauopathy. Frontiers in Immunology, 11. https:\/\/doi.org\/10.3389\/fimmu.2020.00997","DOI":"10.3389\/fimmu.2020.00997"},{"issue":"2","key":"8916_CR17","doi-asserted-by":"publisher","first-page":"29","DOI":"10.3390\/diagnostics8020029","volume":"8","author":"A Khan","year":"2018","unstructured":"Khan, A., & Sergi, C. (2018). Sialidosis: A review of morphology and molecular biology of a rare pediatric disorder. Diagnostics, 8(2), 29. https:\/\/doi.org\/10.3390\/diagnostics8020029","journal-title":"Diagnostics"},{"issue":"4","key":"8916_CR18","doi-asserted-by":"publisher","first-page":"497","DOI":"10.1172\/jci5542","volume":"103","author":"Y Liu","year":"1999","unstructured":"Liu, Y., Wada, R., Kawai, H., Sango, K., Deng, C., Tai, T., McDonald, M. P., Araujo, K., Crawley, J. N., Bierfreund, U., Sandhoff, K., Suzuki, K., & Proia, R. L. (1999). A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder. Journal of Clinical Investigation, 103(4), 497\u2013505. https:\/\/doi.org\/10.1172\/jci5542","journal-title":"Journal of Clinical Investigation"},{"issue":"7","key":"8916_CR19","doi-asserted-by":"publisher","first-page":"1087","DOI":"10.1093\/hmg\/9.7.1087","volume":"9","author":"Y Liu","year":"2000","unstructured":"Liu, Y., Wu, Y. P., Wada, R., Neufeld, E. B., Mullin, K. A., Howard, A. C., Pentchev, P. G., Vanier, M. T., Suzuki, K., & Proia, R. L. (2000). Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann\u2013Pick C disease mouse. Human Molecular Genetics, 9(7), 1087\u20131092. https:\/\/doi.org\/10.1093\/hmg\/9.7.1087","journal-title":"Human Molecular Genetics"},{"issue":"8","key":"8916_CR20","doi-asserted-by":"publisher","first-page":"1495","DOI":"10.1016\/j.ymthe.2019.05.018","volume":"27","author":"J Marshall","year":"2019","unstructured":"Marshall, J., Nietupski, J. B., Park, H., Cao, J., Bangari, D. S., Silvescu, C., Wilper, T., Randall, K., Tietz, D., Wang, B., Ying, X., Leonard, J. P., & Cheng, S. H. (2019). Substrate reduction therapy for Sandhoff disease through inhibition of glucosylceramide synthase activity. Molecular Therapy, 27(8), 1495\u20131506. https:\/\/doi.org\/10.1016\/j.ymthe.2019.05.018","journal-title":"Molecular Therapy"},{"issue":"5","key":"8916_CR21","doi-asserted-by":"publisher","first-page":"781","DOI":"10.1007\/s00702-017-1732-9","volume":"125","author":"AS Mendiola","year":"2018","unstructured":"Mendiola, A. S., & Cardona, A. E. (2018). The IL-1\u03b2 phenomena in neuroinflammatory diseases. Journal of Neural Transmission, 125(5), 781\u2013795. https:\/\/doi.org\/10.1007\/s00702-017-1732-9","journal-title":"Journal of Neural Transmission"},{"issue":"4","key":"8916_CR22","doi-asserted-by":"publisher","first-page":"1902","DOI":"10.1073\/pnas.252784899","volume":"100","author":"K Ohmi","year":"2003","unstructured":"Ohmi, K., Greenberg, D. S., Rajavel, K. S., Ryazantsev, S., Li, H. H., & Neufeld, E. F. (2003). Activated microglia in cortex of mouse models of mucopolysaccharidoses I and IIIB. Proceedings of the National Academy of Sciences, 100(4), 1902\u20131907. https:\/\/doi.org\/10.1073\/pnas.252784899","journal-title":"Proceedings of the National Academy of Sciences"},{"key":"8916_CR23","doi-asserted-by":"publisher","unstructured":"Pelisch, N., Rosas Almanza, J., Stehlik, K. E., Aperi, B. V., & Kroner, A. (2020). CCL3 contributes to secondary damage after spinal cord injury. Journal of Neuroinflammation, 17(1). https:\/\/doi.org\/10.1186\/s12974-020-02037-3","DOI":"10.1186\/s12974-020-02037-3"},{"issue":"1","key":"8916_CR24","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1093\/hmg\/5.1.1","volume":"5","author":"D Phaneuf","year":"1996","unstructured":"Phaneuf, D., Wakamatsu, N., Huang, J. Q., Borowski, A., Peterson, A. C., Fortunato, S. R., Ritter, G., Igdoura, S. A., Morales, C. R., Benoit, G., Akerman, B. R., Leclerc, D., Hanai, N., Marth, J. D., Trasler, J. M., & Gravel, R. A. (1996). Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases. Human Molecular Genetics, 5(1), 1\u201314. https:\/\/doi.org\/10.1093\/hmg\/5.1.1","journal-title":"Human Molecular Genetics"},{"issue":"7","key":"8916_CR25","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1038\/sj.ejhg.5201623","volume":"14","author":"E Piotrowska","year":"2006","unstructured":"Piotrowska, E., Jak\u00f3bkiewicz-Banecka, J., Bara\u0144ska, S., Tylki-Szyma\u0144ska, A., Czartoryska, B., W\u0119grzyn, A., & W\u0119grzyn, G. (2006). Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses. European Journal of Human Genetics, 14(7), 846\u2013852. https:\/\/doi.org\/10.1038\/sj.ejhg.5201623","journal-title":"European Journal of Human Genetics"},{"issue":"11","key":"8916_CR26","doi-asserted-by":"publisher","first-page":"1787","DOI":"10.1161\/atvbaha.109.193268","volume":"29","author":"CA Reichel","year":"2009","unstructured":"Reichel, C. A., Rehberg, M., Lerchenberger, M., Berberich, N., Bihari, P., Khandoga, A. G., Zahler, S., & Krombach, F. (2009). CCL2 and CCL3 mediate neutrophil recruitment via induction of protein synthesis and generation of lipid mediators. Arteriosclerosis Thrombosis and Vascular Biology, 29(11), 1787\u20131793. https:\/\/doi.org\/10.1161\/atvbaha.109.193268","journal-title":"Arteriosclerosis Thrombosis and Vascular Biology"},{"issue":"2","key":"8916_CR27","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1038\/ng1095-170","volume":"11","author":"K Sango","year":"1995","unstructured":"Sango, K., Yamanaka, S., Hoffmann, A., Okuda, Y., Grinberg, A., Westphal, H., McDonald, M. P., Crawley, J. N., Sandhoff, K., Suzuki, K., & Proia, R. L. (1995). Mouse models of Tay\u2013Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism. Nature Genetics, 11(2), 170\u2013176. https:\/\/doi.org\/10.1038\/ng1095-170","journal-title":"Nature Genetics"},{"key":"8916_CR29","doi-asserted-by":"publisher","unstructured":"Seyrantepe, V., Lema, P., Caqueret, A., Dridi, L., Bel Hadj, S., Carpentier, S., Boucher, F., Levade, T., Carmant, L., Gravel, R. A., Hamel, E., Vachon, P., Di Cristo, G., Michaud, J. L., Morales, C. R., & Pshezhetsky, A. V. (2010). Mice doubly-deficient in lysosomal hexosaminidase A and neuraminidase 4 show epileptic crises and rapid neuronal loss. PLoS Genetics, 6(9). https:\/\/doi.org\/10.1371\/journal.pgen.1001118","DOI":"10.1371\/journal.pgen.1001118"},{"key":"8916_CR28","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.expneurol.2017.09.012","volume":"299","author":"V Seyrantepe","year":"2018","unstructured":"Seyrantepe, V., Demir, S. A., Timur, Z. K., Von Gerichten, J., Marsching, C., Erdemli, E., Oztas, E., Takahashi, K., Yamaguchi, K., Ates, N., D\u00f6nmez Demir, B., Dalkara, T., Erich, K., Hopf, C., Sandhoff, R., & Miyagi, T. (2018). Murine sialidase NEU3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease. Experimental Neurology, 299, 26\u201341. https:\/\/doi.org\/10.1016\/j.expneurol.2017.09.012","journal-title":"Experimental Neurology"},{"issue":"13","key":"8916_CR30","doi-asserted-by":"publisher","first-page":"7532","DOI":"10.1073\/pnas.96.13.7532","volume":"96","author":"KA Sheikh","year":"1999","unstructured":"Sheikh, K. A., Sun, J., Liu, Y., Kawai, H., Crawford, T. O., Proia, R. L., Griffin, J. W., & Schnaar, R. L. (1999). Mice lacking complex gangliosides develop Wallerian degeneration and myelination defects. Proceedings of the National Academy of Sciences, 96(13), 7532\u20137537. https:\/\/doi.org\/10.1073\/pnas.96.13.7532","journal-title":"Proceedings of the National Academy of Sciences"},{"issue":"20","key":"8916_CR31","doi-asserted-by":"publisher","first-page":"10662","DOI":"10.1073\/pnas.93.20.10662","volume":"93","author":"K. Takamiya","year":"1996","unstructured":"Takamiya, K., Yamamoto, A., Furukawa, K., Yamashiro, S., Shin, M., Okada, M., Fukumoto, S., Haraguchi, M., Takeda, N., Fujimura, K., Sakae, M., Kishikawa, M., Shiku, H., Furukawa, K., & Aizawa, S. (1996). Mice with disrupted GM2\/GD2 synthase gene lack complex gangliosides but exhibit only subtle defects in their nervous system. Proceedings of the National Academy of Sciences, 93(20), 10662\u201310667. https:\/\/doi.org\/10.1073\/pnas.93.20.10662","journal-title":"Proceedings of the National Academy of Sciences"},{"key":"8916_CR33","doi-asserted-by":"publisher","first-page":"136195","DOI":"10.1016\/j.neulet.2021.136195","volume":"764","author":"C Toro","year":"2021","unstructured":"Toro, C., Zainab, M., & Tifft, C. J. (2021). The GM2 gangliosidoses: Unlocking the mysteries of pathogenesis and treatment. Neuroscience Letters, 764, 136195. https:\/\/doi.org\/10.1016\/j.neulet.2021.136195","journal-title":"Neuroscience Letters"},{"issue":"20","key":"8916_CR34","doi-asserted-by":"publisher","first-page":"10954","DOI":"10.1073\/pnas.97.20.10954","volume":"97","author":"R. Wada","year":"2000","unstructured":"Wada, R., Tifft, C. J., & Proia, R. L. (2000). Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation. Proceedings of the National Academy of Sciences, 97(20), 10954\u201310959. https:\/\/doi.org\/10.1073\/pnas.97.20.10954","journal-title":"Proceedings of the National Academy of Sciences"},{"key":"8916_CR35","doi-asserted-by":"publisher","unstructured":"Wang, C., Zhang, Y., Xu, C., Li, D., Liu, Z., & Wu, Y. (2021). The investigation of genetic and clinical features in patients with hereditary spastic paraplegia in central-southern China. Molecular Genetics & Genomic Medicine, 9(5). https:\/\/doi.org\/10.1002\/mgg3.1627","DOI":"10.1002\/mgg3.1627"},{"issue":"11","key":"8916_CR36","doi-asserted-by":"publisher","first-page":"1723","DOI":"10.3390\/cells11111723","volume":"11","author":"M Wittekindt","year":"2022","unstructured":"Wittekindt, M., Kaddatz, H., Joost, S., Staffeld, A., Bitar, Y., Kipp, M., & Frintrop, L. (2022). Different methods for evaluating microglial activation using anti-ionized calcium-binding adaptor protein-1 immunohistochemistry in the cuprizone model. Cells, 11(11), 1723. https:\/\/doi.org\/10.3390\/cells11111723","journal-title":"Cells"},{"issue":"21","key":"8916_CR37","doi-asserted-by":"publisher","first-page":"9975","DOI":"10.1073\/pnas.91.21.9975","volume":"91","author":"S. Yamanaka","year":"1994","unstructured":"Yamanaka, S., Johnson, M. D., Grinberg, A., Westphal, H., Crawley, J. N., Taniike, M., Suzuki, K., & Proia, R. L. (1994). Targeted disruption of the HEXA gene results in mice with biochemical and pathologic features of Tay-Sachs disease. Proceedings of the National Academy of Sciences, 91(21), 9975\u20139979. https:\/\/doi.org\/10.1073\/pnas.91.21.9975","journal-title":"Proceedings of the National Academy of Sciences"},{"issue":"1","key":"8916_CR38","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1074\/jbc.273.1.66","volume":"273","author":"JA Yuziuk","year":"1998","unstructured":"Yuziuk, J. A., Bertoni, C., Beccari, T., Orlacchio, A., Wu, Y. Y., Li, S. C., & Li, Y. T. (1998). Specificity of mouse GM2 activator protein and \u03b2-N-acetylhexosaminidases A and B. Journal of Biological Chemistry, 273(1), 66\u201372. https:\/\/doi.org\/10.1074\/jbc.273.1.66","journal-title":"Journal of Biological Chemistry"},{"issue":"6","key":"8916_CR39","doi-asserted-by":"publisher","first-page":"1365","DOI":"10.1016\/j.jhep.2014.07.006","volume":"61","author":"X Zhang","year":"2014","unstructured":"Zhang, X., Shen, J., Man, K., Chu, E. S. H., Yau, T. O., Sung, J. C. Y., Go, M. Y. Y., Deng, J., Lu, L., Wong, V. W. S., Sung, J. J. Y., Farrell, G., & Yu, J. (2014). CXCL10 plays a key role as an inflammatory mediator and a non-invasive biomarker of non-alcoholic steatohepatitis. Journal of Hepatology, 61(6), 1365\u20131375. https:\/\/doi.org\/10.1016\/j.jhep.2014.07.006","journal-title":"Journal of Hepatology"}],"container-title":["NeuroMolecular Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12017-026-08916-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12017-026-08916-x","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12017-026-08916-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T19:41:18Z","timestamp":1781638878000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12017-026-08916-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3,9]]},"references-count":38,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,12]]}},"alternative-id":["8916"],"URL":"https:\/\/doi.org\/10.1007\/s12017-026-08916-x","relation":{},"ISSN":["1559-1174"],"issn-type":[{"value":"1559-1174","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3,9]]},"assertion":[{"value":"4 January 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 February 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 March 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"16"}}