{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T17:55:15Z","timestamp":1790358915852,"version":"4.1.0"},"reference-count":115,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T00:00:00Z","timestamp":1770076800000},"content-version":"vor","delay-in-days":948,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2023,6,21]],"date-time":"2023-06-21T00:00:00Z","timestamp":1687305600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Hereditary ataxias are a large group of neurodegenerative diseases that have cerebellar or spinocerebellar dysfunction as core feature, occurring as an isolated sign or as part of a syndrome. Based on neuropathology, this group of diseases has so far been classified into cerebellar cortical degenerations, spinocerebellar degenerations, cerebellar ataxias without substantial neurodegeneration, canine multiple system degeneration, and episodic ataxia. Several new hereditary ataxia syndromes are described, but most of these diseases have similar clinical signs and unspecific diagnostic findings, wherefore achieving a definitive diagnosis in these dogs is challenging. Eighteen new genetic variants associated with these diseases have been discovered in the last decade, allowing clinicians to reach a definitive diagnosis for most of these conditions, and allowing breeding schemes to adapt to prevent breeding of affected puppies. This review summarizes the current knowledge about hereditary ataxias in dogs, and proposes to add a \u201cmultifocal degenerations with predominant (spino)cerebellar component\u201d category regrouping canine multiple system degeneration, new hereditary ataxia syndromes that do not fit in 1 of the previous categories, as well as specific neuroaxonal dystrophies and lysosomal storage diseases that cause major (spino)cerebellar dysfunction.<\/jats:p>","DOI":"10.1111\/jvim.16742","type":"journal-article","created":{"date-parts":[[2023,6,21]],"date-time":"2023-06-21T09:50:54Z","timestamp":1687341054000},"page":"1306-1322","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Phenotypic and genetic aspects of hereditary ataxia in dogs"],"prefix":"10.1093","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5208-2605","authenticated-orcid":false,"given":"Kimberley","family":"Stee","sequence":"first","affiliation":[{"name":"Ghent University Small Animal Department, Faculty of Veterinary Medicine, , Merelbeke,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4316-8984","authenticated-orcid":false,"given":"Mario","family":"Van Poucke","sequence":"additional","affiliation":[{"name":"Faculty of Veterinary Sciences, Ghent University Department of Veterinary and Biosciences, , Merelbeke,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4993-589X","authenticated-orcid":false,"given":"Mark","family":"Lowrie","sequence":"additional","affiliation":[{"name":"Dovecote Veterinary Hospital , Derby,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Van Ham","sequence":"additional","affiliation":[{"name":"Faculty of Veterinary Medicine, Ghent University Small Animal Department, , Merelbeke,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc","family":"Peelman","sequence":"additional","affiliation":[{"name":"Faculty of Veterinary Sciences, Ghent University Department of Veterinary and Biosciences, , Merelbeke,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1349-3484","authenticated-orcid":false,"given":"Natasha","family":"Olby","sequence":"additional","affiliation":[{"name":"North Carolina State University Department of Clinical Sciences, , Raleigh, North Carolina,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofie F.M.","family":"Bhatti","sequence":"additional","affiliation":[{"name":"Faculty of Veterinary Medicine, Ghent University Small Animal Department, , Merelbeke,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,7,1]]},"reference":[{"key":"2026020302581114800_jvim16742-bib-0001","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1016\/j.cvsm.2014.07.005","article-title":"Canine hereditary ataxia","volume":"44","author":"Urkasemsin","year":"2014","journal-title":"Vet Clin North Am Small"},{"issue":"4","key":"2026020302581114800_jvim16742-bib-0002","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1007\/s12311-019-01052-2","article-title":"The classification of autosomal recessive cerebellar ataxias: a consensus statement from the Society for Research on the cerebellum and ataxias task force","volume":"18","author":"Beaudin","year":"2019","journal-title":"Cerebellum"},{"key":"2026020302581114800_jvim16742-bib-0003","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1002\/mds.27415","article-title":"The genetic nomenclature of recessive cerebellar ataxias","volume":"33","author":"Rossi","year":"2018","journal-title":"Mov Disord"},{"key":"2026020302581114800_jvim16742-bib-0004","first-page":"374","article-title":"Veterinary Neuroanatomy and Clinical Neurology","author":"de Lahunta","year":"2021","edition":"5th ed."},{"key":"2026020302581114800_jvim16742-bib-0005","first-page":"65","article-title":"Abiotrophy in domestic animals: a review","volume":"54","author":"De Lahunta","year":"1990","journal-title":"Can Vet J"},{"key":"2026020302581114800_jvim16742-bib-0006","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1186\/1471-2156-13-55","article-title":"Genome-wide mRNA sequencing of a single canine cerebellar cortical degeneration case leads to the identification of a disease associated SPTBN2 mutation","volume":"13","author":"Forman","year":"2012","journal-title":"BMC Genet"},{"key":"2026020302581114800_jvim16742-bib-0007","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1002759","article-title":"A SEL1L mutation links a canine progressive early-onset cerebellar ataxia to the endoplasmic reticulum-associated protein degradation (ERAD) machinery","volume":"8","author":"Ky\u00f6stil\u00e4","year":"2012","journal-title":"PLoS Genet"},{"key":"2026020302581114800_jvim16742-bib-0008","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1003991","article-title":"Canine hereditary ataxia in Old English Sheepdog and Gordon Setters in associated with a defect in the autophagy gene encoding RAB24","volume":"10","author":"Agler","year":"2014","journal-title":"PLoS Genet"},{"key":"2026020302581114800_jvim16742-bib-0009","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1186\/s12863-016-0433-y","article-title":"Genome sequencing reveals a splice donor site mutation in the SNX14 gene associated with a novel cerebellar cortical degeneration in the Hungarian Vizsla dog breed","volume":"17","author":"Fenn","year":"2016","journal-title":"BMC Genet"},{"key":"2026020302581114800_jvim16742-bib-0010","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1111\/jvim.14738","article-title":"Genetics of hereditary ataxia in Scottish Terriers","volume":"31","author":"Urkasemsin","year":"2017","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0011","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1111\/j.1365-2052.2011.02199.x","article-title":"Mapping cerebellar abiotrophy in Australian Kelpies","volume":"42","author":"Shearman","year":"2011","journal-title":"Anim Genet"},{"key":"2026020302581114800_jvim16742-bib-0012","doi-asserted-by":"crossref","first-page":"1709","DOI":"10.3390\/genes13101709","article-title":"Cerebellar abiotrophy in Australian working Kelpies is associated with two major risk loci","volume":"13","author":"Wade","year":"2022","journal-title":"Genes"},{"key":"2026020302581114800_jvim16742-bib-0013","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0064627","article-title":"Missense mutation in CAPN1 is associated with spinocerebellar ataxia in the Parson Russell Terrier Dog Breed","volume":"8","author":"Forman","year":"2013","journal-title":"PLoS One"},{"key":"2026020302581114800_jvim16742-bib-0014","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1111\/jvim.12355","article-title":"A homozygous KCNJ10 mutation in Jack Russell terriers and related breeds with spinocerebellar ataxia with myokymia, seizures or both","volume":"28","author":"Gilliam","year":"2014","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0015","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1186\/s13028-015-0115-1","article-title":"A KCNJ10 mutation previously identified in the Russell group of terriers also occurs in smooth-haired fox terriers with hereditary ataxia and related breeds","volume":"57","author":"Rohdin","year":"2015","journal-title":"Acta Vet Scand"},{"key":"2026020302581114800_jvim16742-bib-0016","article-title":"Neuromyotonia, myokimia and spinocerebellar ataxia in two Patterdale terrier littermates","volume":"10","author":"Fisher","year":"2022","journal-title":"Vet Rec"},{"key":"2026020302581114800_jvim16742-bib-0017","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1111\/j.1748-5827.2011.01123.x","article-title":"Neuromyotonia in a dachshund with clinical and electrophysiological signs of spinocerebellar ataxia","volume":"52","author":"Vanhaesebrouck","year":"2011","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0018","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1038\/ejhg.2016.157","article-title":"The novel homozygous KCNJ10 c.986T&gt;C (p.Leu329Pro) variant is pathogenic for the SeSAME\/EAST homologue in Malinois dogs","volume":"25","author":"van Poucke","year":"2017","journal-title":"Eur J Hum Genet"},{"key":"2026020302581114800_jvim16742-bib-0019","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1534\/g3.116.038455","article-title":"A missense variant in KCNJ10 in Belgian shepherd dogs affected by spongy degeneration with cerebellar ataxia (SDCA1)","volume":"7","author":"Mauri","year":"2017","journal-title":"Genes Genom Genet"},{"key":"2026020302581114800_jvim16742-bib-0020","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1038\/s41431-019-0432-3","article-title":"Truncating SLC12A6 variants cause different clinical phenotypes in humans and dogs","volume":"27","author":"van Poucke","year":"2019","journal-title":"Eur J Hum Genet"},{"key":"2026020302581114800_jvim16742-bib-0021","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1111\/jvim.16594","article-title":"Spinocerebellar ataxia in the Bouvier des Ardennes breed is caused by a KCNJ10 missense variant","volume":"37","author":"Stee","year":"2023","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0022","doi-asserted-by":"crossref","first-page":"362","DOI":"10.3390\/genes10050362","article-title":"A missense variant in SCN8A in alpine Dachsbracke dogs affected by spinocerebellar ataxia","volume":"10","author":"Letko","year":"2019","journal-title":"Genes"},{"key":"2026020302581114800_jvim16742-bib-0023","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1111\/j.1939-1676.2010.0666.x","article-title":"A truncated retrotransposon disrupts the GRM1 coding sequence in Coton de Tulear dogs with Bandera's neonatal ataxia","volume":"25","author":"Zeng","year":"2011","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0024","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1007\/s00335-014-9547-6","article-title":"Spinocerebellar ataxia in the Italian Spinone dog is associated with an intronic GAA repeat expansion in ITPR1","volume":"26","author":"Forman","year":"2015","journal-title":"Mamm Genome"},{"key":"2026020302581114800_jvim16742-bib-0025","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1008527","article-title":"Characterisation of canine KCNIP4: a novel gene for cerebellar ataxia identified by whole-genome sequencing two affected Norwegian Buhund dogs","volume":"16","author":"Jenkins","year":"2020","journal-title":"PLoS Genet"},{"key":"2026020302581114800_jvim16742-bib-0026","article-title":"Canine multiple system degeneration is associated with distinct SERAC1 mutations in two different dog breeds. In: Proceedings of the 63rd Annual Meeting of the American Society of Human Genetics; October 22-26, 2013; Boston, USA","author":"Guo"},{"key":"2026020302581114800_jvim16742-bib-0027","doi-asserted-by":"crossref","first-page":"2729","DOI":"10.1534\/g3.117.043018","article-title":"A SINE insertion in ATP1B2 in Belgian shepherd dogs affected by spongy degeneration with cerebellar ataxia (SDCA2)","volume":"7","author":"Mauri","year":"2017","journal-title":"Genes Genom Genet"},{"issue":"4","key":"2026020302581114800_jvim16742-bib-0028","article-title":"Deletion of the SELENOP gene leads to CNS atrophy with cerbellar ataxia in dogs","volume":"17","author":"Christen","year":"2021","journal-title":"PLoS Genet"},{"key":"2026020302581114800_jvim16742-bib-0029","first-page":"1","article-title":"A PNPLA8 frameshift variant in Australian shepherd dogs with hereditary ataxia","volume":"00","author":"Abitbol","year":"2022","journal-title":"Anim Genet"},{"issue":"4","key":"2026020302581114800_jvim16742-bib-0030","article-title":"A 1bp deletion in HACE1 causes ataxia in Norwegian elkhound, black","volume":"17","author":"Bellamy","year":"2022","journal-title":"PLoS One"},{"key":"2026020302581114800_jvim16742-bib-0031","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.3390\/genes13071223","article-title":"SLC25A12 missense variant in Nova Scotia duck tolling retrievers affected by cerebellar degeneration-myositis complex (CDMC)","volume":"13","author":"Christen","year":"2022","journal-title":"Genes"},{"key":"2026020302581114800_jvim16742-bib-0032","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1005169","article-title":"A missense change in the ATG4D gene links abberant autophagy to a neurodegenerative vacuolar storage disease","volume":"11","author":"Ky\u00f6stil\u00e4","year":"2015","journal-title":"PLoS Genet"},{"key":"2026020302581114800_jvim16742-bib-0033","doi-asserted-by":"crossref","first-page":"2773","DOI":"10.1534\/g3.118.200376","article-title":"A missense mutation in the vacuolar protein sorting 11 (VPS11) gene is associated with neuroaxonal dystrophy in Rottweiler dogs","volume":"8","author":"Lucot","year":"2018","journal-title":"Genes Genom Genet"},{"key":"2026020302581114800_jvim16742-bib-0034","doi-asserted-by":"crossref","first-page":"14775","DOI":"10.1073\/pnas.0914206107","article-title":"A canine arylsulfatase G (ARSG) mutation leading to a sulfatase deficiency is associated with neuronal ceroid lipofuscinosis","volume":"107","author":"Abitbol","year":"2010","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2026020302581114800_jvim16742-bib-0035","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.ymgme.2005.11.005","article-title":"A mutation in the cathepsin D gene (CTSD) in American Bulldogs with neuronal ceroid lipofuscinosis","volume":"87","author":"Awano","year":"2006","journal-title":"Mol Genet Metab"},{"key":"2026020302581114800_jvim16742-bib-0036","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1023\/A:1005630013448","article-title":"Isolation and characterization of the normal canine \u00df-galactosidase gene and its mutation in a dog model of GM1-gangliosidosis","volume":"23","author":"Wang","year":"2000","journal-title":"J Inherit Metab Dis"},{"issue":"4","key":"2026020302581114800_jvim16742-bib-0037","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology","volume":"17","author":"Richards","year":"2015","journal-title":"Genet Med"},{"key":"2026020302581114800_jvim16742-bib-0038","first-page":"619","article-title":"Postnatal cerebellar cortical degeneration in Labrador retriever puppies","volume":"32","author":"Perille","year":"2001","journal-title":"Can Vet J"},{"key":"2026020302581114800_jvim16742-bib-0039","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1111\/j.1939-1676.1994.tb03207.x","article-title":"Cerebellar Purkinje cell degeneration and coat color dilution in a family of Rhodesian Ridgeback dogs","volume":"8","author":"Chieffo","year":"1994","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0040","first-page":"570","article-title":"Cerebellar cortical degeneration in a Labrador Retriever","volume":"36","author":"Bildfell","year":"1995","journal-title":"Can Vet J"},{"key":"2026020302581114800_jvim16742-bib-0041","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1053\/j.tcam.2017.06.003","article-title":"Cerebellar cortical abiotrophy in young Labrador-retrievers","volume":"32","author":"Sen","year":"2017","journal-title":"Top Companion Anim Med"},{"key":"2026020302581114800_jvim16742-bib-0042","doi-asserted-by":"crossref","first-page":"432","DOI":"10.1111\/avj.12211","article-title":"Late onset cerebellar abiotrophy in a Labrador retriever","volume":"92","author":"Bertalan","year":"2014","journal-title":"Aust Vet J"},{"key":"2026020302581114800_jvim16742-bib-0043","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1111\/j.1748-5827.2005.tb00323.x","article-title":"Cerebellar cortical degeneration in three English bulldogs: clinical and neuropathological findings","volume":"46","author":"Gandini","year":"2005","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0044","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1080\/01652176.2000.9695050","article-title":"Cerebellar cortical abiotrophy in two Portuguese Podenco littermates","volume":"22","author":"van Tongeren","year":"2000","journal-title":"Vet Quart"},{"key":"2026020302581114800_jvim16742-bib-0045","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1111\/j.1748-5827.2008.00616.x","article-title":"Cerebellar cortical degeneration in two Azores cattle dog littermates","volume":"49","author":"Varej\u00e3o","year":"2008","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0046","first-page":"657","article-title":"Cerebellar abiotrophy in a miniature schnauzer","volume":"44","author":"Berry","year":"2003","journal-title":"Can Vet J"},{"key":"2026020302581114800_jvim16742-bib-0047","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1292\/jvms.69.1047","article-title":"Clinicopathological features of canine neuroaxonal dystrophy and cerebellar cortical abiotrophy in Papillon and Papillon-related dogs","volume":"69","author":"Nibe","year":"2007","journal-title":"J Vet Med Sci"},{"key":"2026020302581114800_jvim16742-bib-0048","doi-asserted-by":"crossref","DOI":"10.4061\/2010\/406275","article-title":"Late onset of cerebellar abiotrophy in a boxer dog","volume":"2010","author":"Gumber","year":"2010","journal-title":"Vet Med Int"},{"key":"2026020302581114800_jvim16742-bib-0049","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1111\/j.1748-5827.2006.00298.x","article-title":"Cerebellar cortical abiotrophy in Lagotto Romagnolo dogs","volume":"48","author":"Jokinen","year":"2007","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0050","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.2460\/javma.1996.208.08.1277","article-title":"Clinical, hematologic and biochemical features of a syndrome in Bernese Mountain dogs characterized by hepatocerebellar degeneration","volume":"208","author":"Carmichael","year":"1996","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0051","first-page":"79","article-title":"Inherited cerebellar degeneration in the rough coated collie","volume":"8","author":"Hartley","year":"1978","journal-title":"Aust Vet Pract"},{"key":"2026020302581114800_jvim16742-bib-0052","first-page":"176","article-title":"Preliminary characterization of a cerebellar ataxia in Jack Russell terriers","volume":"10","author":"Coates","year":"1996","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0053","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1080\/00480169.1980.34737","article-title":"Cerebellar degeneration in the Border Collie","volume":"28","author":"Gill","year":"1980","journal-title":"N Zeal Vet J"},{"key":"2026020302581114800_jvim16742-bib-0054","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1354\/vp.39-6-736","article-title":"Cerebellar abiotrophy in a family of Border Collie dogs","volume":"39","author":"Sandy","year":"2002","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0055","first-page":"55","article-title":"Cerebellar granuloprival degeneration in an Australian kelpie and a Labrador retriever dog","volume":"54","author":"Huska","year":"2013","journal-title":"Can Vet J"},{"key":"2026020302581114800_jvim16742-bib-0056","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1111\/j.1748-5827.2006.00257.x","article-title":"Cerebellar cortical degeneration with selective granule cell loss in Bavarian Mountain dogs","volume":"46","author":"Flegel","year":"2007","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0057","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1016\/S0165-5728(00)00330-1","article-title":"Presumed immune-mediated cerebellar granuloprival degeneration in the Coton de Tul\u00e9ar breed","volume":"110","author":"Tipold","year":"2000","journal-title":"J Neuroimmunol"},{"key":"2026020302581114800_jvim16742-bib-0058","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1046\/j.1439-0442.2002.00495.x","article-title":"Cerebellar granuloprival degeneration in an Italian hound","volume":"49","author":"Cantile","year":"2002","journal-title":"J Vet Med A Physiol Pathol Clin Med"},{"key":"2026020302581114800_jvim16742-bib-0059","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1007\/s004010050865","article-title":"Late-onset progressive spinocerebellar degeneration in Brittany spaniel dogs","volume":"96","author":"Higgins","year":"1998","journal-title":"Acta Neuropathol"},{"key":"2026020302581114800_jvim16742-bib-0060","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1111\/j.1939-1676.1994.tb03235.x","article-title":"A tremor syndrome with central axonopathy in Scottish terriers","volume":"8","author":"van Ham","year":"1994","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0061","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1111\/j.1748-5827.2010.00955.x","article-title":"Episodic cerebellar dysfunction in a bichon frise: a canine case of episodic ataxia?","volume":"51","author":"Hopkins","year":"2010","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0062","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1080\/00480169.1982.34897","article-title":"Suspected inherited cerebellar neuroaxonal dystrophy in collie sheep dogs","volume":"30","author":"Clark","year":"1982","journal-title":"N Zeal Vet J"},{"key":"2026020302581114800_jvim16742-bib-0063","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1186\/s13028-016-0218-3","article-title":"Pathological findings in a dachshund-cross dog with neuroaxonal dystrophy","volume":"58","author":"Pintus","year":"2016","journal-title":"Acta Vet Scand"},{"key":"2026020302581114800_jvim16742-bib-0064","first-page":"299","article-title":"Practical Guide to Canine and Feline Neurology","author":"Dewey","year":"2016","edition":"3rd ed."},{"key":"2026020302581114800_jvim16742-bib-0065","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1007\/BF00690384","article-title":"An adult case of canine neuronal ceroid lipofuscinosis","volume":"39","author":"Cummings","year":"1977","journal-title":"Acta Neuropathol"},{"key":"2026020302581114800_jvim16742-bib-0066","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1177\/030098588902600203","article-title":"Canine GM2 Gangliosidosis: morphological and biochemical analysis","volume":"26","author":"Singer","year":"1989","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0067","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1002\/ana.410090512","article-title":"Canine inherited ataxia","volume":"9","author":"Cork","year":"1981","journal-title":"Ann Neurol"},{"key":"2026020302581114800_jvim16742-bib-0068","doi-asserted-by":"crossref","first-page":"886","DOI":"10.2460\/javma.1981.179.09.886","article-title":"Clinical features of inherited cerebellar degeneration in Gordon Setters","volume":"179","author":"Steinberg","year":"1981","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0069","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1177\/030098588802500412","article-title":"Cerebellar cortical degeneration in Beagle Dogs","volume":"25","author":"Yasuba","year":"1988","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0070","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1111\/j.1748-5827.2000.tb03210.x","article-title":"Cerebellar cortical abiotrophy in a beagle","volume":"41","author":"Kent","year":"2000","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0071","doi-asserted-by":"crossref","first-page":"1162","DOI":"10.2460\/javma.2000.217.1162","article-title":"Cerebellar degeneration in old English sheepdogs","volume":"217","author":"Steinberg","year":"2000","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0072","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.tvjl.2004.08.015","article-title":"Neurodegenerative diseases in domestic animals, a comparative review","volume":"171","author":"Sis\u00f3","year":"2006","journal-title":"Vet J"},{"issue":"4","key":"2026020302581114800_jvim16742-bib-0073","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1111\/j.1740-8261.2009.01655.x","article-title":"Development of a morphometric magnetic resonance image parameter suitable for distinguishing between normal dogs and dogs with cerebellar atrophy","volume":"51","author":"Thames","year":"2010","journal-title":"Vet Radiol Ultrasound"},{"key":"2026020302581114800_jvim16742-bib-0074","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1111\/j.1748-5827.2001.tb02491.x","article-title":"Diagnosis of cerebellar cortical degeneration in a Scottish terrier using magnetic resonance imaging","volume":"42","author":"van der Merwe","year":"2001","journal-title":"J Small Anim Pract"},{"key":"2026020302581114800_jvim16742-bib-0075","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1111\/j.1939-1676.2010.0499.x","article-title":"Hereditary cerebellar degeneration in Scottish Terriers","volume":"24","author":"Urkasemsin","year":"2010","journal-title":"J Vet Intern Med"},{"issue":"4","key":"2026020302581114800_jvim16742-bib-0076","first-page":"852","article-title":"Mapping of Purkinje neuron loss and polyglucosan body accumulatin in hereditary cerebellar degeneration in Scottish Terriers","volume":"49","author":"Urkasemsin","year":"2011","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0077","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1111\/j.1751-0813.1989.tb13959.x","article-title":"Hereditary cerebellar abiotrophy in Australian Kelpie dogs","volume":"66","author":"Thomas","year":"1989","journal-title":"Aust Vet J"},{"key":"2026020302581114800_jvim16742-bib-0078","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1111\/j.1365-2052.2008.01753.x","article-title":"Elimination of SETX, SYNE1 and ATCAY as the cause of cerebellar abiotrophy in the Australian Kelpies","volume":"39","author":"Shearman","year":"2008","journal-title":"Anim Genet"},{"key":"2026020302581114800_jvim16742-bib-0079","first-page":"871","article-title":"Hereditary ataxia in smooth-haired fox terriers","volume":"69","author":"Bj\u00f6rck","year":"1957","journal-title":"Vet Rec"},{"key":"2026020302581114800_jvim16742-bib-0080","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/BF00685524","article-title":"Ataxia in Jack Russell Terriers","volume":"26","author":"Hartley","year":"1973","journal-title":"Acta Neuropathol"},{"key":"2026020302581114800_jvim16742-bib-0081","first-page":"769","article-title":"\u2018Continuous muscle fibre activity\u2019 in six dogs with episodic myokymia, stiffness and collapse","volume":"155","author":"van Ham","year":"2004","journal-title":"Vet Rec"},{"key":"2026020302581114800_jvim16742-bib-0082","first-page":"515","article-title":"Hereditary ataxia in the Jack Russell Terrier \u2013 clinical and genetic investigations","volume":"18","author":"Wessmann","year":"2004","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0083","doi-asserted-by":"crossref","first-page":"882","DOI":"10.1111\/j.1939-1676.2010.0525.x","article-title":"Clinical and electrophysiological characterization of myokymia and neuromyotonia in Jack Russell Terriers","volume":"24","author":"Vanhaesebrouck","year":"2010","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0084","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.tvjl.2010.07.011","article-title":"Myokymia and neuromyotonia in 37 Jack Russel terriers","volume":"189","author":"Bhatti","year":"2011","journal-title":"Vet J"},{"key":"2026020302581114800_jvim16742-bib-0085","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1136\/vr.e3642","article-title":"Hereditary ataxia in Jack Russel terriers in the UK","volume":"170","author":"Simpson","year":"2012","journal-title":"Vet Rec"},{"key":"2026020302581114800_jvim16742-bib-0086","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/j.tvjl.2013.03.002","article-title":"Myokymia and neuromyotonia in veterinary medicine: a comparison with peripheral nerve hyperexcitability syndrome in humans","volume":"197","author":"Vanhaesebrouck","year":"2013","journal-title":"Vet J"},{"key":"2026020302581114800_jvim16742-bib-0087","first-page":"45","article-title":"Hereditary ataxia in fox terriers","volume":"1","author":"Bj\u00f6rck","year":"1962","journal-title":"Acta Neuropathol"},{"key":"2026020302581114800_jvim16742-bib-0088","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1136\/vr.b4821","article-title":"New aspects of hereditary ataxia in smooth-haired fox terriers","volume":"166","author":"Rohdin","year":"2010","journal-title":"Vet Rec"},{"key":"2026020302581114800_jvim16742-bib-0089","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1111\/j.1939-1676.2011.0720.x","article-title":"Spongy degeneration with cerebellar ataxia in Malinois puppies: a hereditary autosomal recessive disorder?","volume":"25","author":"Kleiter","year":"2011","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0090","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1186\/s12917-016-0862-x","article-title":"Genome-wide association study for hereditary ataxia in the parson Russell terrier and DNA-testing for ataxia-associated mutations in the parson and Jack Russell terrier","volume":"12","author":"Gast","year":"2016","journal-title":"BMC Vet Res"},{"key":"2026020302581114800_jvim16742-bib-0091","article-title":"Association of spinocerebellar ataxia related variants with myokimia and neuromyotonia in dogs","author":"Vanhaesebrouck","year":"2022","journal-title":"BioRxiv"},{"key":"2026020302581114800_jvim16742-bib-0092","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.jcpa.2017.10.011","article-title":"Genetic variants in KCNJ10 and ATP1B2 in Malinois puppies with spongy degeneration and cerebellar ataxia","volume":"158","author":"H\u00f6gler","year":"2018","journal-title":"J Comp Pathol"},{"key":"2026020302581114800_jvim16742-bib-0093","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1111\/j.1939-1676.2002.tb02408.x","article-title":"Neonatal cerebellar ataxia in coton de tulear dogs","volume":"16","author":"Coates","year":"2002","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0094","doi-asserted-by":"crossref","first-page":"774","DOI":"10.2460\/javma.253.6.774","article-title":"Hereditary ataxia in four related Norwegian Buhunds","volume":"253","author":"Mari","year":"2018","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0095","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.2460\/javma.1976.168.12.1119","article-title":"Hereditary cerebellar cortical and extrapyramidal nuclear abiotrophy in Kerry blue terriers","volume":"168","author":"de Lahunta","year":"1976","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0096","first-page":"198","article-title":"Hereditary cerebellar neuronal Abiotrophy in a Kerry blue terrier dog","volume":"19","author":"Deforest","year":"1978","journal-title":"Can Vet J"},{"key":"2026020302581114800_jvim16742-bib-0097","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1177\/030098588302000202","article-title":"Hereditary striatonigral and cerebello-olivary degeneration of the Kerry blue terrier","volume":"20","author":"Montgomery","year":"1983","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0098","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1093\/jhered\/esi086","article-title":"Genetic mapping of canine multiple system degeneration and ectodermal dysplasia loci","volume":"96","author":"O'Brien","year":"2005","journal-title":"J Hered"},{"key":"2026020302581114800_jvim16742-bib-0099","doi-asserted-by":"crossref","first-page":"10672","DOI":"10.1074\/jbc.M117.783068","article-title":"The phospholipase iPLA2\u03b3 is a major mediator releasing oxidized aliphatic chains from cardiolipin, integrating mitochondrial bioenergetics and signaling","volume":"292","author":"Liu","year":"2017","journal-title":"J Biol Chem"},{"key":"2026020302581114800_jvim16742-bib-0100","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1177\/0300985817712793","article-title":"Basal autophagy is altered in Lagotto Romagnolo dogs with an ATG4D mutation","volume":"54","author":"Pernilla","year":"2017","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0101","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1097\/00005072-198305000-00006","article-title":"Canine Neuroaxonal dystrophy","volume":"42","author":"Cork","year":"1983","journal-title":"J Neuropathol Exp Neurol"},{"key":"2026020302581114800_jvim16742-bib-0102","doi-asserted-by":"crossref","first-page":"464","DOI":"10.2460\/javma.1984.184.04.464","article-title":"Neuroaxonal dystrophy of Rottweiler dogs","volume":"184","author":"Chrisman","year":"1984","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0103","doi-asserted-by":"crossref","first-page":"1560","DOI":"10.2460\/javma.1988.192.11.1560","article-title":"Neuroaxonal dystrophy in a Rottweiler pup","volume":"192","author":"Evans","year":"1988","journal-title":"J Am Vet Med Assoc"},{"key":"2026020302581114800_jvim16742-bib-0104","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1007\/s004010100386","article-title":"Juvenile neuroaxonal dystrophy in a Rottweiler: accumulation of synaptic proteins in dystrophic axons","volume":"102","author":"Sis\u00f3","year":"2001","journal-title":"Acta Neuropathol"},{"key":"2026020302581114800_jvim16742-bib-0105","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1111\/j.1939-1676.2004.tb00161.x","article-title":"Cerebellar cortical degeneration in adult American Staffordshire terriers","volume":"18","author":"Olby","year":"2004","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0106","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1556\/avet.2012.054","article-title":"Magnetic resonance imaging and brainstem auditory evoked responses in the diagnosis of cerebellar cortical degeneration in American Staffordshire terriers","volume":"61","author":"Kwiatkowska","year":"2013","journal-title":"Acta Vet Hung"},{"key":"2026020302581114800_jvim16742-bib-0107","first-page":"518","article-title":"Cerebellar cortical degeneration in an American Staffordshire terrier","volume":"131","author":"Buijtels","year":"2006","journal-title":"Tijdschr Diegeneeskd"},{"key":"2026020302581114800_jvim16742-bib-0108","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1007\/s00401-004-0902-7","article-title":"Adult onset thalamocerebellar degeneration in dogs associated to neuronal storage of ceroid lipopigment","volume":"108","author":"Sis\u00f3","year":"2004","journal-title":"Acta Neuropathol"},{"key":"2026020302581114800_jvim16742-bib-0109","first-page":"369","article-title":"Cerebellar cortical abiotrophy in American Staffordshire terriers: clinical and pathological description of 3 cases","volume":"145","author":"Hanzl\u00edcek","year":"2003","journal-title":"Schweiz Arch Tierheilkd"},{"key":"2026020302581114800_jvim16742-bib-0110","doi-asserted-by":"crossref","first-page":"431","DOI":"10.15654\/TPK-150766","article-title":"Neuronal ceroid lipofuscinosis in an adult American Staffordshire terrier","volume":"44","author":"Nolte","year":"2016","journal-title":"Tierarztl Prax Ausg K Kleintiere Heimtiere"},{"key":"2026020302581114800_jvim16742-bib-0111","doi-asserted-by":"crossref","first-page":"10310","DOI":"10.1073\/pnas.1202071109","article-title":"Arylsulfatase G inactivation causes loss of heparan sulfate 3-0-sulfatase activity and mucopolysaccharidosis in mice","volume":"109","author":"Kowalewski","year":"2012","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2026020302581114800_jvim16742-bib-0112","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1111\/j.1939-1676.2005.tb02657.x","article-title":"A variant form of neuronal ceroid lipofuscinosis in American bulldogs","volume":"19","author":"Evans","year":"2005","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0113","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/j.1939-1676.1989.tb00320.x","article-title":"Neuronal-visceral GM1 gangliosidosis in Portugese water dogs","volume":"3","author":"Shell","year":"1989","journal-title":"J Vet Intern Med"},{"key":"2026020302581114800_jvim16742-bib-0114","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1177\/030098588802500403","article-title":"GM1 Gangliosidosis in Portuguese water dogs: pathologic and biochemical findings","volume":"25","author":"Saunders","year":"1988","journal-title":"Vet Pathol"},{"key":"2026020302581114800_jvim16742-bib-0115","first-page":"675","article-title":"Canine GM1-gangliosidosis. A clinical, morphologic, histochemical and biochemical comparison of two different models","volume":"140","author":"Alroy","year":"1992","journal-title":"Am J Pathol"}],"container-title":["Journal of Veterinary Internal Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jvim\/article-pdf\/37\/4\/1306\/66658043\/jvim16742.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jvim\/article-pdf\/37\/4\/1306\/66658043\/jvim16742.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T07:58:29Z","timestamp":1770105509000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jvim\/article\/37\/4\/1306\/8447910"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,1]]},"references-count":115,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2023,7,1]]}},"URL":"https:\/\/doi.org\/10.1111\/jvim.16742","relation":{},"ISSN":["1939-1676"],"issn-type":[{"value":"1939-1676","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,7]]},"published":{"date-parts":[[2023,7,1]]},"assertion":[{"value":"2022-11-16","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-07","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-06-21","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}