{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,10]],"date-time":"2026-05-10T09:13:12Z","timestamp":1778404392023,"version":"3.51.4"},"reference-count":83,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,1,1]],"date-time":"2024-01-01T00:00:00Z","timestamp":1704067200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,11,28]],"date-time":"2023-11-28T00:00:00Z","timestamp":1701129600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2024,1]]},"DOI":"10.1016\/j.nbd.2023.106368","type":"journal-article","created":{"date-parts":[[2023,11,29]],"date-time":"2023-11-29T18:35:49Z","timestamp":1701282949000},"page":"106368","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":7,"special_numbering":"C","title":["Antisense oligonucleotide-mediated disruption of HTT caspase-6 cleavage site ameliorates the phenotype of YAC128 Huntington disease mice"],"prefix":"10.1016","volume":"190","author":[{"given":"Elsa C.","family":"Kuijper","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maurice","family":"Overzier","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ernst","family":"Suidgeest","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Oleh","family":"Dzyubachyk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C\u00e9cile","family":"Maguin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Baptiste","family":"P\u00e9rot","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julien","family":"Flament","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yavuz","family":"Ariyurek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hailiang","family":"Mei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ronald A.M.","family":"Buijsen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Louise","family":"van der Weerd","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Willeke","family":"van Roon-Mom","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2023.106368_bb0005","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.bbr.2015.06.011","article-title":"The light spot test: measuring anxiety in mice in an automated home-cage environment","volume":"294","author":"Aarts","year":"2015","journal-title":"Behav. Brain Res."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0010","doi-asserted-by":"crossref","first-page":"2937","DOI":"10.1038\/s41467-018-05347-6","article-title":"Sensitive and powerful single-cell RNA sequencing using mcSCRB-seq","volume":"9","author":"Bagnoli","year":"2018","journal-title":"Nat. Commun."},{"issue":"9","key":"10.1016\/j.nbd.2023.106368_bb0015","doi-asserted-by":"crossref","first-page":"5136","DOI":"10.1007\/s00415-022-11187-8","article-title":"Cerebrospinal fluid levels of proenkephalin and prodynorphin are differentially altered in Huntington\u2019s and Parkinson\u2019s disease","volume":"269","author":"Barschke","year":"2022","journal-title":"J. Neurol."},{"key":"10.1016\/j.nbd.2023.106368_bb0020","first-page":"141","article-title":"Emerging therapies for Huntington\u2019s disease - focus on N-terminal huntingtin and huntingtin exon 1","volume":"16","author":"van der Bent","year":"2022","journal-title":"Biologics."},{"issue":"9","key":"10.1016\/j.nbd.2023.106368_bb0025","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0075099","article-title":"Striatal pre-enkephalin overexpression improves Huntington\u2019s disease symptoms in the R6\/2 mouse model of Huntington\u2019s disease","volume":"8","author":"Bissonnette","year":"2013","journal-title":"PloS One"},{"issue":"2\u20133","key":"10.1016\/j.nbd.2023.106368_bb0030","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.brainresbull.2010.05.005","article-title":"Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL\/6J) Huntington\u2019s disease transgenic mice","volume":"88","author":"Brooks","year":"2012","journal-title":"Brain Res. Bull."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0035","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1523\/JNEUROSCI.4178-07.2008","article-title":"A transcriptome database for astrocytes, neurons, and oligodendrocytes: a new resource for understanding brain development and function","volume":"28","author":"Cahoy","year":"2008","journal-title":"J. Neurosci."},{"issue":"12","key":"10.1016\/j.nbd.2023.106368_bb0040","first-page":"1963","volume":"11","author":"Callens","year":"2022","journal-title":"Dysregulated Ca2+ Homeostasis as a Central Theme in Neurodegeneration: Lessons from Alzheimer’s Disease and Wolfram Syndrome."},{"issue":"4","key":"10.1016\/j.nbd.2023.106368_bb0045","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1002\/jnr.490400404","article-title":"Effects of carbonic anhydrase II (CAII) deficiency on CNS structure and function in the myelin-deficient CAII-deficient double mutant mouse","volume":"40","author":"Cammer","year":"1995","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.nbd.2023.106368_bb0050","series-title":"Antisense Drug Technology Principles, Strategies, and Applications","article-title":"Manufacturing and analytical processes for 2\u2032-O-(2-Methoxyethyl)-modified oligonucleotides","author":"Capaldi","year":"2007"},{"key":"10.1016\/j.nbd.2023.106368_bb0055","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.biopha.2016.09.007","article-title":"In vivo proof-of-concept of removal of the huntingtin caspase cleavage motif-encoding exon 12 approach in the YAC128 mouse model of Huntington\u2019s disease","volume":"84","author":"Casaca-Carreira","year":"2016","journal-title":"Biomedicine & Pharmacotherapy = Biomedecine & Pharmacotherapie."},{"key":"10.1016\/j.nbd.2023.106368_bb0060","doi-asserted-by":"crossref","first-page":"24","DOI":"10.3389\/fninf.2020.00024","article-title":"Sammba-MRI: A Library for Processing SmAll-MaMmal BrAin MRI Data in Python","volume":"14","author":"Celestine","year":"2020","journal-title":"Front. Neuroinform."},{"key":"10.1016\/j.nbd.2023.106368_bb0065","doi-asserted-by":"crossref","first-page":"132","DOI":"10.3389\/fncel.2014.00132","article-title":"The sodium leak channel, NALCN, in health and disease","volume":"8","author":"Cochet-Bissuel","year":"2014","journal-title":"Front. Cell. Neurosci."},{"issue":"3","key":"10.1016\/j.nbd.2023.106368_bb0070","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1006\/cbmr.1996.0014","article-title":"AFNI: software for analysis and visualization of functional magnetic resonance neuroimages","volume":"29","author":"Cox","year":"1996","journal-title":"Computers Biomed. Res. Int. J."},{"issue":"7","key":"10.1016\/j.nbd.2023.106368_bb0075","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1006846","article-title":"Elimination of huntingtin in the adult mouse leads to progressive behavioral deficits, bilateral thalamic calcification, and altered brain iron homeostasis","volume":"13","author":"Dietrich","year":"2017","journal-title":"PLoS Genet."},{"issue":"5334","key":"10.1016\/j.nbd.2023.106368_bb0080","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0085","first-page":"160","volume":"42","author":"Dzyubachyk","year":"2015","journal-title":"Super-resolution reconstruction of late gadolinium-enhanced MRI for improved myocardial scar assessment."},{"issue":"9","key":"10.1016\/j.nbd.2023.106368_bb0090","first-page":"1295","article-title":"Activation of Caspase-6 is promoted by a mutant huntingtin fragment and blocked by an allosteric inhibitor compound. Cell","volume":"26","author":"Ehrnhoefer","year":"2019","journal-title":"Chem. Biol."},{"issue":"17","key":"10.1016\/j.nbd.2023.106368_bb0095","doi-asserted-by":"crossref","first-page":"2255","DOI":"10.15252\/embj.201490808","article-title":"Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation","volume":"34","author":"El-Daher","year":"2015","journal-title":"EMBO J."},{"issue":"2\u20133","key":"10.1016\/j.nbd.2023.106368_bb0100","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1159\/000065698","article-title":"Transferrin regulates transcription of the MBP gene and its action synergizes with IGF-1 to enhance myelinogenesis in the md rat","volume":"24","author":"Espinosa-Jeffrey","year":"2002","journal-title":"Dev. Neurosci."},{"key":"10.1016\/j.nbd.2023.106368_bb0105","doi-asserted-by":"crossref","first-page":"500","DOI":"10.3389\/fncel.2019.00500","article-title":"Brainiac caspases: beyond the wall of apoptosis","volume":"13","author":"Espinosa-Oliva","year":"2019","journal-title":"Front. Cell. Neurosci."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0110","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1089\/nat.2013.0452","article-title":"Preventing formation of toxic N-terminal huntingtin fragments through antisense oligonucleotide-mediated protein modification","volume":"24","author":"Evers","year":"2014","journal-title":"Nucleic Acid Ther."},{"key":"10.1016\/j.nbd.2023.106368_bb0115","series-title":"Huntington\u2019s Disease","first-page":"97","article-title":"Mouse models of Huntington\u2019s disease","author":"Farshim","year":"2018"},{"issue":"8","key":"10.1016\/j.nbd.2023.106368_bb0120","first-page":"2085","volume":"289","author":"Fatoba","year":"2022","journal-title":"Complement cascade functions during brain development and neurodegeneration."},{"issue":"8","key":"10.1016\/j.nbd.2023.106368_bb0125","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1074\/mcp.RA120.002102","article-title":"Changes in the oligodendrocyte progenitor cell proteome with ageing","volume":"19","author":"de la Fuente","year":"2020","journal-title":"Mol. Cell. Proteomic. MCP."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0130","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1038\/s41598-017-00684-w","article-title":"Overexpression of GSN could decrease inflammation and apoptosis in EAE and may enhance vitamin D therapy on EAE\/MS","volume":"7","author":"Gao","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2023.106368_bb0135","doi-asserted-by":"crossref","first-page":"423","DOI":"10.3389\/fnmol.2017.00423","article-title":"IL-33 provides neuroprotection through suppressing apoptotic, Autophagic and NF-\u03baB-mediated inflammatory pathways in a rat model of recurrent neonatal seizure","volume":"10","author":"Gao","year":"2017","journal-title":"Front. Mol. Neurosci."},{"issue":"W1","key":"10.1016\/j.nbd.2023.106368_bb0140","doi-asserted-by":"crossref","first-page":"W515","DOI":"10.1093\/nar\/gkaa306","article-title":"GeneTrail 3: advanced high-throughput enrichment analysis","volume":"48","author":"Gerstner","year":"2020","journal-title":"Nucleic Acids Res."},{"issue":"2","key":"10.1016\/j.nbd.2023.106368_bb0145","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1016\/0306-4522(96)00295-3","article-title":"Preferential localization of annexin V to the axon terminal","volume":"75","author":"Gotow","year":"1996","journal-title":"Neuroscience."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0150","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1016\/j.cell.2006.04.026","article-title":"Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin","volume":"125","author":"Graham","year":"2006","journal-title":"Cell."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0155","first-page":"50","volume":"65","author":"Jansen","year":"2017","journal-title":"Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0160","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.1016\/j.stemcr.2022.04.006","article-title":"Iron accumulation induces oxidative stress, while depressing inflammatory polarization in human iPSC-derived microglia","volume":"17","author":"Kenkhuis","year":"2022","journal-title":"Stem Cell Rep."},{"issue":"17","key":"10.1016\/j.nbd.2023.106368_bb0165","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.154108","article-title":"A pathogenic proteolysis-resistant huntingtin isoform induced by an antisense oligonucleotide maintains huntingtin function","volume":"7","author":"Kim","year":"2022","journal-title":"JCI Insight."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0170","doi-asserted-by":"crossref","first-page":"412","DOI":"10.1038\/d41573-021-00088-6","article-title":"Double setback for ASO trials in Huntington disease","volume":"20","author":"Kingwell","year":"2021","journal-title":"Nat. Rev. Drug Discov."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0175","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1016\/S0092-8674(03)00719-0","article-title":"Involvement of histone H1.2 in apoptosis induced by DNA double-strand breaks","volume":"114","author":"Konishi","year":"2003","journal-title":"Cell."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0180","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1016\/j.neuron.2012.05.009","article-title":"Sustained therapeutic reversal of Huntington\u2019s disease by transient repression of huntingtin synthesis","volume":"74","author":"Kordasiewicz Holly","year":"2012","journal-title":"Neuron."},{"issue":"4","key":"10.1016\/j.nbd.2023.106368_bb0185","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1002\/npr2.12208","article-title":"Brain-derived neurotrophic factor predominantly regulates the expression of synapse-related genes in the striatum: insights from in vitro transcriptomics","volume":"41","author":"Koshimizu","year":"2021","journal-title":"Neuropsychopharmacol. Rep."},{"issue":"4","key":"10.1016\/j.nbd.2023.106368_bb0190","doi-asserted-by":"crossref","first-page":"2532","DOI":"10.1007\/s12035-021-02680-8","article-title":"Huntington disease gene expression signatures in blood compared to brain of YAC128 mice as candidates for monitoring of pathology","volume":"59","author":"Kuijper","year":"2022","journal-title":"Mol. Neurobiol."},{"issue":"12","key":"10.1016\/j.nbd.2023.106368_bb0195","doi-asserted-by":"crossref","first-page":"8808","DOI":"10.1074\/jbc.M109.075028","article-title":"Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease","volume":"285","author":"Landles","year":"2010","journal-title":"J. Biol. Chem."},{"issue":"7124","key":"10.1016\/j.nbd.2023.106368_bb0200","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1038\/nature05453","article-title":"Genome-wide atlas of gene expression in the adult mouse brain","volume":"445","author":"Lein","year":"2007","journal-title":"Nature."},{"issue":"10","key":"10.1016\/j.nbd.2023.106368_bb0205","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pbio.1002282","article-title":"Decreased SGK1 expression and function contributes to behavioral deficits induced by traumatic stress","volume":"13","author":"Licznerski","year":"2015","journal-title":"PLoS Biol."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0210","doi-asserted-by":"crossref","first-page":"7791","DOI":"10.1038\/s41467-022-35388-x","article-title":"Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation","volume":"13","author":"Lim","year":"2022","journal-title":"Nat. Commun."},{"issue":"3","key":"10.1016\/j.nbd.2023.106368_bb0215","doi-asserted-by":"crossref","first-page":"885","DOI":"10.4049\/jimmunol.1600259","article-title":"Characterization of the expression and function of the C-type lectin receptor CD302 in mice and humans reveals a role in dendritic cell migration","volume":"197","author":"Lo","year":"2016","journal-title":"J. Immunol."},{"issue":"3","key":"10.1016\/j.nbd.2023.106368_bb0220","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbc.2022.101671","article-title":"Structural insights into the interactions and epigenetic functions of human nucleic acid repair protein ALKBH6","volume":"298","author":"Ma","year":"2022","journal-title":"J. Biol. Chem."},{"issue":"4","key":"10.1016\/j.nbd.2023.106368_bb0225","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1038\/cdd.2014.163","article-title":"Regulation of neuronal survival and morphology by the E3 ubiquitin ligase RNF157","volume":"22","author":"Matz","year":"2015","journal-title":"Cell Death Differentiation."},{"issue":"3","key":"10.1016\/j.nbd.2023.106368_bb0230","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.nbd.2009.05.007","article-title":"Systematic behavioral evaluation of Huntington\u2019s disease transgenic and knock-in mouse models","volume":"35","author":"Menalled","year":"2009","journal-title":"Neurobiol. Dis."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0235","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1523\/JNEUROSCI.21-06-01830.2001","article-title":"Tissue-specific proteolysis of huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington\u2019s disease striatum","volume":"21","author":"Mende-Mueller","year":"2001","journal-title":"J. Neurosci."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0240","first-page":"106","article-title":"Integrin beta 4 (ITGB4) and its tyrosine-1510 phosphorylation promote pancreatic tumorigenesis and regulate the MEK1-ERK1\/2 signaling pathway","volume":"20","author":"Meng","year":"2020","journal-title":"Bosn. J. Basic Med. Sci."},{"key":"10.1016\/j.nbd.2023.106368_bb0245","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1007\/978-1-0716-2010-6_23","article-title":"Delivery of antisense oligonucleotides to the mouse brain by Intracerebroventricular injections","volume":"2434","author":"Metz","year":"2022","journal-title":"Methods in Molecular Biology (Clifton, NJ)."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0250","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1038\/s41598-017-01510-z","article-title":"The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington\u2019s disease patients","volume":"7","author":"Neueder","year":"2017","journal-title":"Sci. Rep."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0255","doi-asserted-by":"crossref","first-page":"91","DOI":"10.31887\/DCNS.2016.18.1\/pnopoulos","article-title":"Huntington disease: a single-gene degenerative disorder of the striatum","volume":"18","author":"Nopoulos","year":"2016","journal-title":"Dialogues Clin. Neurosci."},{"key":"10.1016\/j.nbd.2023.106368_bb0260","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.jneumeth.2013.09.021","article-title":"RATS: rapid automatic tissue segmentation in rodent brain MRI","volume":"221","author":"Oguz","year":"2014","journal-title":"J. Neurosci. Methods"},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0265","doi-asserted-by":"crossref","first-page":"5","DOI":"10.3892\/br.2022.1587","article-title":"Recent approaches on Huntington\u2019s disease (review)","volume":"18","author":"Palaiogeorgou","year":"2023","journal-title":"Biomed Rep."},{"issue":"21","key":"10.1016\/j.nbd.2023.106368_bb0270","doi-asserted-by":"crossref","first-page":"3581","DOI":"10.1093\/hmg\/ddac036","article-title":"Longitudinal multimodal MRI characterization of a knock-in mouse model of Huntington\u2019s disease reveals early gray and white matter alterations","volume":"31","author":"P\u00e9rot","year":"2022","journal-title":"Hum. Mol. Genet."},{"issue":"Pt 4","key":"10.1016\/j.nbd.2023.106368_bb0275","first-page":"919","article-title":"Prevention of depressive behaviour in the YAC128 mouse model of Huntington disease by mutation at residue 586 of huntingtin","volume":"132","author":"Pouladi","year":"2009","journal-title":"Brain."},{"issue":"8","key":"10.1016\/j.nbd.2023.106368_bb0280","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.1093\/hmg\/ddq026","article-title":"Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression","volume":"19","author":"Pouladi","year":"2010","journal-title":"Hum. Mol. Genet."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0285","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1111\/bpa.12426","article-title":"Huntington\u2019s disease (HD): the neuropathology of a multisystem neurodegenerative disorder of the human brain","volume":"26","author":"R\u00fcb","year":"2016","journal-title":"Brain pathology (Zurich, Switzerland)."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0290","doi-asserted-by":"crossref","first-page":"2366","DOI":"10.1073\/pnas.1221891110","article-title":"Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease","volume":"110","author":"Sathasivam","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"13","key":"10.1016\/j.nbd.2023.106368_bb0295","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","article-title":"Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease","volume":"12","author":"Slow","year":"2003","journal-title":"Hum. Mol. Genet."},{"issue":"6","key":"10.1016\/j.nbd.2023.106368_bb0300","first-page":"1115","article-title":"A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles","volume":"26","author":"Southwell","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2023.106368_bb0305","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1016\/j.bbr.2015.09.004","article-title":"The FVB\/N mice: a well suited strain to study learning and memory processes using olfactory cues","volume":"296","author":"St\u00e9phane","year":"2016","journal-title":"Behav. Brain Res."},{"issue":"7","key":"10.1016\/j.nbd.2023.106368_bb0310","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1016\/S1474-4422(22)00121-1","article-title":"Potential disease-modifying therapies for Huntington\u2019s disease: lessons learned and future opportunities","volume":"21","author":"Tabrizi","year":"2022","journal-title":"Lancet Neurol."},{"issue":"14","key":"10.1016\/j.nbd.2023.106368_bb0315","doi-asserted-by":"crossref","first-page":"2770","DOI":"10.1093\/hmg\/ddr176","article-title":"Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative","volume":"20","author":"Tebbenkamp","year":"2011","journal-title":"Hum. Mol. Genet."},{"issue":"13","key":"10.1016\/j.nbd.2023.106368_bb0320","first-page":"2621","article-title":"Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease","volume":"25","author":"Teo","year":"2016","journal-title":"Hum. Mol. Genet."},{"issue":"2","key":"10.1016\/j.nbd.2023.106368_bb0325","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1089\/nat.2017.0705","article-title":"Intracerebroventricular administration of a 2\u2019-O-methyl Phosphorothioate antisense oligonucleotide results in activation of the innate immune system in mouse brain","volume":"28","author":"Toonen","year":"2018","journal-title":"Nucleic acid therapeutics."},{"issue":"5","key":"10.1016\/j.nbd.2023.106368_bb0330","doi-asserted-by":"crossref","DOI":"10.1016\/j.mcpro.2023.100534","article-title":"Proteomic Analysis of Huntington’s Disease Medium Spiny Neurons Identifies Alterations in Lipid Droplets","volume":"22","author":"Tshilenge","year":"2023","journal-title":"Mol. Cell. Proteomics"},{"issue":"9","key":"10.1016\/j.nbd.2023.106368_bb0335","doi-asserted-by":"crossref","first-page":"1954","DOI":"10.1093\/hmg\/dds005","article-title":"Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice","volume":"21","author":"Uribe","year":"2012","journal-title":"Hum. Mol. Genet."},{"issue":"9","key":"10.1016\/j.nbd.2023.106368_bb0340","doi-asserted-by":"crossref","first-page":"1513","DOI":"10.1093\/hmg\/ddl072","article-title":"Body weight is modulated by levels of full-length huntingtin","volume":"15","author":"Van Raamsdonk","year":"2006","journal-title":"Hum. Mol. Genet."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0345","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.nbd.2006.12.010","article-title":"Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain","volume":"26","author":"Van Raamsdonk","year":"2007","journal-title":"Neurobiol. Dis."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0350","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1523\/JNEUROSCI.1305-11.2012","article-title":"Transgenic mouse model expressing the caspase 6 fragment of mutant huntingtin","volume":"32","author":"Waldron-Roby","year":"2012","journal-title":"J. Neurosci."},{"issue":"17","key":"10.1016\/j.nbd.2023.106368_bb0355","doi-asserted-by":"crossref","first-page":"2738","DOI":"10.1093\/hmg\/ddn175","article-title":"Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington\u2019s disease","volume":"17","author":"Wang","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2023.106368_bb0360","series-title":"Functional Network of the Long Non-coding RNA Growth Arrest-Specific Transcript 5 and Its Interacting Proteins in Senescence","first-page":"12","author":"Wang","year":"2021"},{"issue":"4","key":"10.1016\/j.nbd.2023.106368_bb0365","first-page":"1806","article-title":"Transferrin is an insulin-like growth factor-binding Protein-3 binding Protein1","volume":"86","author":"Weinzimer","year":"2001","journal-title":"J. Clin. Endocrinol. Metabol."},{"issue":"18","key":"10.1016\/j.nbd.2023.106368_bb0370","doi-asserted-by":"crossref","first-page":"7862","DOI":"10.1523\/JNEUROSCI.22-18-07862.2002","article-title":"Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington\u2019s disease","volume":"22","author":"Wellington","year":"2002","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2023.106368_bb0375","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2020.104963","article-title":"The contribution of glial cells to Huntington\u2019s disease pathogenesis","volume":"143","author":"Wilton","year":"2020","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2023.106368_bb0380","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.nbd.2014.12.030","article-title":"Partial rescue of some features of Huntington disease in the genetic absence of caspase-6 in YAC128 mice","volume":"76","author":"Wong","year":"2015","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2023.106368_bb0385","series-title":"Identification of Key Biomarkers and Pathways for Maintaining Cognitively Normal Brain Aging Based on Integrated Bioinformatics Analysis","volume":"14","author":"Xu","year":"2022"},{"issue":"12","key":"10.1016\/j.nbd.2023.106368_bb0390","doi-asserted-by":"crossref","first-page":"2023","DOI":"10.1109\/TMI.2010.2058861","article-title":"LOGISMOS\u2014layered optimal graph image segmentation of multiple objects and surfaces: cartilage segmentation in the knee joint","volume":"29","author":"Yin","year":"2010","journal-title":"IEEE Trans. Med. Imaging"},{"issue":"2","key":"10.1016\/j.nbd.2023.106368_bb0395","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1038\/ng1095-155","article-title":"Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington\u2019s disease gene homologue","volume":"11","author":"Zeitlin","year":"1995","journal-title":"Nat. Genet."},{"issue":"1","key":"10.1016\/j.nbd.2023.106368_bb0400","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1016\/j.bbrc.2018.02.070","article-title":"GAS5 silencing protects against hypoxia\/ischemia-induced neonatal brain injury","volume":"497","author":"Zhao","year":"2018","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2023.106368_bb0405","article-title":"Human ALKBH6 is required for maintenance of genomic stability and promoting cell survival during exposure of alkylating agents in pancreatic Cancer","volume":"12","author":"Zhao","year":"2021","journal-title":"Front. Genet."},{"key":"10.1016\/j.nbd.2023.106368_bb0410","first-page":"9","article-title":"Gab1 mediates PDGF signaling and is essential to oligodendrocyte differentiation and CNS myelination","author":"Zhou","year":"2020","journal-title":"eLife."},{"issue":"9","key":"10.1016\/j.nbd.2023.106368_bb0415","first-page":"4675","volume":"22","author":"Ziabska","year":"2021","journal-title":"Aberrant Complement System Activation in Neurological Disorders."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996123003844?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996123003844?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:39:23Z","timestamp":1760852363000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996123003844"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1]]},"references-count":83,"alternative-id":["S0969996123003844"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2023.106368","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2024,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Antisense oligonucleotide-mediated disruption of HTT caspase-6 cleavage site ameliorates the phenotype of YAC128 Huntington disease mice","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2023.106368","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2023 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"106368"}}