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Select item 109403051.

Lowering mutant huntingtin by small molecules relieves Huntington’s disease symptoms and progression

Anat Bahat, Elad Itzhaki, Benjamin Weiss, Michael Tolmasov, Michael Tsoory, Yael Kuperman, Alexander Brandis, Khriesto A Shurrush, Rivka Dikstein

EMBO Mol Med. 2024 Mar; 16(3): 523–546. Published online 2024 Feb 19. doi: 10.1038/s44321-023-00020-y

PMCID:: PMC10940305

Article PDF–3.3M

Select item 106683792.

Naïve Huntington’s disease microglia mount a normal response to inflammatory stimuli but display a partially impaired development of innate immune tolerance that can be counteracted by ganglioside GM1

Noam Steinberg, Danny Galleguillos, Asifa Zaidi, Melanie Horkey, Simonetta Sipione

J Neuroinflammation. 2023; 20: 276. Published online 2023 Nov 23. doi: 10.1186/s12974-023-02963-y

PMCID:: PMC10668379

Article PDF–6.2M

Select item 106251273.

A Specific Mini‐Intrabody Mediates Lysosome Degradation of Mutant Huntingtin

Caijuan Li, Yingqi Lin, Yizhi Chen, Xichen Song, Xiao Zheng, Jiawei Li, Jun He, Xiusheng Chen, Chunhui Huang, Wei Wang, Jianhao Wu, Jiaxi Wu, Jiale Gao, Zhuchi Tu, Xiao‐Jiang Li, Sen Yan, Shihua Li

Adv Sci (Weinh) 2023 Nov; 10(31): 2301120. Published online 2023 Sep 8. doi: 10.1002/advs.202301120

PMCID:: PMC10625127

Article PDF–5.0M

Select item 106070774.

Small Molecule Pytren-4QMn Metal Complex Slows down Huntington’s Disease Progression in Male zQ175 Transgenic Mice

Marián Merino, Sonia González, Mª Carmen Tronch, Ana Virginia Sánchez-Sánchez, Mª Paz Clares, Antonio García-España, Enrique García-España, José L. Mullor

Int J Mol Sci. 2023 Oct; 24(20): 15153. Published online 2023 Oct 13. doi: 10.3390/ijms242015153

PMCID:: PMC10607077

Article PDF–1.7M

Select item 105080005.

Comparison of Huntington’s disease phenotype progression in male and female heterozygous FDNQ175 mice

Si Han Li, Tash-Lynn L. Colson, Jingwei Chen, Khaled S. Abd-Elrahman, Stephen S. G. Ferguson

Mol Brain. 2023; 16: 67. Published online 2023 Sep 19. doi: 10.1186/s13041-023-01054-6

PMCID:: PMC10508000

Article PDF–7.1M

Select item 104886836.

Huntingtin loss in hepatocytes is associated with altered metabolism, adhesion, and liver zonation

Robert M Bragg, Sydney R Coffey, Jeffrey P Cantle, Shikai Hu, Sucha Singh, Samuel RW Legg, Cassandra A McHugh, Amreen Toor, Scott O Zeitlin, Seung Kwak, David Howland, Thomas F Vogt, Satdarshan P Monga, Jeffrey B Carroll

Life Sci Alliance. 2023 Nov; 6(11): e202302098. Published online 2023 Sep 8. doi: 10.26508/lsa.202302098

PMCID:: PMC10488683

Article PDF–3.2M

Select item 104704687.

Altered exocytosis of inhibitory synaptic vesicles at single presynaptic terminals of cultured striatal neurons in a knock-in mouse model of Huntington’s disease

Chen Xu, Sidong Chen, Xingxiang Chen, Ka Hei Ho, Chungwon Park, Hanna Yoo, Suk-Ho Lee, Hyokeun Park

Front Mol Neurosci. 2023; 16: 1175522. Published online 2023 Aug 17. doi: 10.3389/fnmol.2023.1175522

PMCID:: PMC10470468

Article PDF–3.2M

Select item 103619938.

Amyloid modifier SERF1a interacts with polyQ-expanded huntingtin-exon 1 via helical interactions and exacerbates polyQ-induced toxicity

Tien-Ying Tsai, Chun-Yu Chen, Tien-Wei Lin, Tien-Chang Lin, Feng-Lan Chiu, Orion Shih, Ming-Yun Chang, Yu-Chun Lin, An-Chung Su, Chiung-Mei Chen, U-Ser Jeng, Hung-Chih Kuo, Chi-Fon Chang, Yun-Ru Chen

Commun Biol. 2023; 6: 767. Published online 2023 Jul 21. doi: 10.1038/s42003-023-05142-0

Correction in:: Commun Biol. 2023; 6: 1021.

PMCID:: PMC10361993

Article PDF–4.7M

Select item 103419189.

Cell-Type Specific Regulation of Cholesterogenesis by CYP46A1 Re-Expression in zQ175 HD Mouse Striatum

Katleen Pinchaud, Chloé Masson, Baptiste Dayre, Coline Mounier, Jean-François Gilles, Peter Vanhoutte, Jocelyne Caboche, Sandrine Betuing

Int J Mol Sci. 2023 Jul; 24(13): 11001. Published online 2023 Jul 2. doi: 10.3390/ijms241311001

PMCID:: PMC10341918

Article PDF–4.3M

Select item 1023225310.

AAV5-miHTT-mediated huntingtin lowering improves brain health in a Huntington’s disease mouse model

Sarah B Thomson, Anouk Stam, Cynthia Brouwers, Valentina Fodale, Alberto Bresciani, Michael Vermeulen, Sara Mostafavi, Terri L Petkau, Austin Hill, Andrew Yung, Bretta Russell-Schulz, Piotr Kozlowski, Alex MacKay, Da Ma, Mirza Faisal Beg, Melvin M Evers, Astrid Vallès, Blair R Leavitt

Brain. 2023 Jun; 146(6): 2298–2315. Published online 2022 Dec 12. doi: 10.1093/brain/awac458

PMCID:: PMC10232253

Article PDF–1.1M

Select item 1017558111.

Striatal spatial heterogeneity, clustering, and white matter association of GFAP⁺ astrocytes in a mouse model of Huntington’s disease

Taylor G. Brown, Mackenzie N. Thayer, Jillian G. VanTreeck, Nicole Zarate, Damyan W. Hart, Sarah Heilbronner, Rocio Gomez-Pastor

Front Cell Neurosci. 2023; 17: 1094503. Published online 2023 Apr 28. doi: 10.3389/fncel.2023.1094503

PMCID:: PMC10175581

Article PDF–6.2M

Select item 989816012.

Neuronal and astrocytic contributions to Huntington’s disease dissected with zinc finger protein transcriptional repressors

Mohitkumar R. Gangwani, Joselyn S. Soto, Yasaman Jami-Alahmadi, Srushti Tiwari, Riki Kawaguchi, James A. Wohlschlegel, Baljit S. Khakh

Cell Rep. Author manuscript; available in PMC 2023 Feb 4.

Published in final edited form as: Cell Rep. 2023 Jan 31; 42(1): 111953. Published online 2023 Jan 7. doi: 10.1016/j.celrep.2022.111953

PMCID:: PMC9898160

Article PDF–4.4M

Select item 984536213.

Transcriptional vulnerabilities of striatal neurons in human and rodent models of Huntington’s disease

Ayano Matsushima, Sergio Sebastian Pineda, Jill R. Crittenden, Hyeseung Lee, Kyriakitsa Galani, Julio Mantero, Geoffrey Tombaugh, Manolis Kellis, Myriam Heiman, Ann M. Graybiel

Nat Commun. 2023; 14: 282. Published online 2023 Jan 17. doi: 10.1038/s41467-022-35752-x

PMCID:: PMC9845362

Article PDF–4.1M

Select item 979406914.

Reduction in PA28αβ activation in HD mouse brain correlates to increased mHTT aggregation in cell models

Karlijne W. Geijtenbeek, Jolien Janzen, Aleksandra E. Bury, Alicia Sanz-Sanz, Ron A. Hoebe, Marie K. Bondulich, Gillian P. Bates, Eric A. J. Reits, Sabine Schipper-Krom

PLoS One. 2022; 17(12): e0278130. Published online 2022 Dec 27. doi: 10.1371/journal.pone.0278130

PMCID:: PMC9794069

Article PDF–2.1M

Select item 971479115.

Benefits of global mutant huntingtin lowering diminish over time in a Huntington’s disease mouse model

Deanna M. Marchionini, Jeh-Ping Liu, Alberto Ambesi-Impiombato, Kimberly Kerker, Kim Cirillo, Mukesh Bansal, Rich Mushlin, Daniela Brunner, Sylvie Ramboz, Mei Kwan, Kirsten Kuhlbrodt, Karsten Tillack, Finn Peters, Leena Rauhala, John Obenauer, Jonathan R. Greene, Christopher Hartl, Vinod Khetarpal, Brenda Lager, Jim Rosinski, Jeff Aaronson, Morshed Alam, Ethan Signer, Ignacio Muñoz-Sanjuán, David Howland, Scott O. Zeitlin

JCI Insight. 2022 Oct 24; 7(20): e161769. Published online 2022 Oct 24. doi: 10.1172/jci.insight.161769

PMCID:: PMC9714791

Article PDF–4.9M

Select item 953626316.

A pathogenic proteolysis–resistant huntingtin isoform induced by an antisense oligonucleotide maintains huntingtin function

Hyeongju Kim, Sophie Lenoir, Angela Helfricht, Taeyang Jung, Zhana K. Karneva, Yejin Lee, Wouter Beumer, Geert B. van der Horst, Herma Anthonijsz, Levi C.M. Buil, Frits van der Ham, Gerard J. Platenburg, Pasi Purhonen, Hans Hebert, Sandrine Humbert, Frédéric Saudou, Pontus Klein, Ji-Joon Song

JCI Insight. 2022 Sep 8; 7(17): e154108. Published online 2022 Sep 8. doi: 10.1172/jci.insight.154108

PMCID:: PMC9536263

Article PDF–10M

Select item 891738217.

Suppression of toxicity of the mutant huntingtin protein by its interacting compound, desonide

Haikun Song, Cen Wang, Chenggang Zhu, Ziying Wang, Huiya Yang, Peng Wu, Xiaotian Cui, Juan Botas, Yongjun Dang, Yu Ding, Yiyan Fei, Boxun Lu

Proc Natl Acad Sci U S A. 2022 Mar 8; 119(10): e2114303119. Published online 2022 Mar 1. doi: 10.1073/pnas.2114303119

PMCID:: PMC8917382

Article PDF–2.9M

Select item 865791518.

C57BL/6 Background Attenuates mHTT Toxicity in the Striatum of YAC128 Mice

Michaela K. Back, Johanna Kurzawa, Sonia Ruggieri, Jakob von Engelhardt

Int J Mol Sci. 2021 Dec; 22(23): 12664. Published online 2021 Nov 23. doi: 10.3390/ijms222312664

PMCID:: PMC8657915

Article PDF–2.6M

Select item 863412019.

Huntingtin silencing delays onset and slows progression of Huntington’s disease: a biomarker study

Hongshuai Liu, Chuangchuang Zhang, Jiadi Xu, Jing Jin, Liam Cheng, Xinyuan Miao, Qian Wu, Zhiliang Wei, Peiying Liu, Hanzhang Lu, Peter C M van Zijl, Christopher A Ross, Jun Hua, Wenzhen Duan

Brain. 2021 Oct; 144(10): 3101–3113. Published online 2021 Mar 27. doi: 10.1093/brain/awab190

PMCID:: PMC8634120

Article PDF–977K

Select item 860969220.

Identification of Full-Length Wild-Type and Mutant Huntingtin Interacting Proteins by Crosslinking Immunoprecipitation in Mice Brain Cortex

Karen A. Sap, Arzu Tugce Guler, Aleksandra Bury, Dick Dekkers, Jeroen A.A. Demmers, Eric A. Reits

J Huntingtons Dis. 2021; 10(3): 335–347. Prepublished online 2021 Jun 12. Published online 2021 Sep 29. doi: 10.3233/JHD-210476

PMCID:: PMC8609692

Article PDF–927K

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