{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T08:36:23Z","timestamp":1793435783652,"version":"4.5.0"},"reference-count":35,"publisher":"Wildlife Disease Association","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Wildlife Diseases"],"published-print":{"date-parts":[[2021,10,11]]},"abstract":"<jats:p>A lysosomal storage disease, identified as a mucopolysaccharidosis (MPS), was diagnosed in a free-living Kaka (Nestor meridionalis), an endemic New Zealand parrot, which exhibited weakness, incoordination, and seizures. Histopathology showed typical colloid-like cytoplasmic inclusions in Purkinje cells and many other neurons throughout the brain. Electron microscopy revealed that storage bodies contained a variety of linear, curved, or circular membranous profiles and electron-dense bodies. Because the bird came from a small isolated population of Kaka in the northern South Island, a genetic cause was deemed likely. Tandem mass spectrometry revealed increased levels of heparan sulfate-derived disaccharides in the brain and liver compared with tissues from controls. Enzymatic assays documented low levels of iduronate-2-sulfatase activity, which causes a lysosomal storage disorder called MPS type II or Hunter syndrome. A captive breeding program is currently in progress, and the possibility of detecting carriers of this disorder warrants further investigation.<\/jats:p>","DOI":"10.7589\/jwd-d-20-00173","type":"journal-article","created":{"date-parts":[[2021,10,11]],"date-time":"2021-10-11T01:42:56Z","timestamp":1633916576000},"page":"884-890","source":"Crossref","is-referenced-by-count":2,"title":["MUCOPOLYSACCHARIDOSIS II (MPS II) IN A FREE-LIVING KAKA (NESTOR MERIDIONALIS) IN NEW ZEALAND"],"prefix":"10.7589","volume":"57","author":[{"given":"Robert D.","family":"Jolly","sequence":"first","affiliation":[{"name":"School of Veterinary Science, Massey University, Palmerston North 4442, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stuart A.","family":"Hunter","sequence":"additional","affiliation":[{"name":"School of Veterinary Science, Massey University, Palmerston North 4442, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurice R.","family":"Alley","sequence":"additional","affiliation":[{"name":"School of Veterinary Science, Massey University, Palmerston North 4442, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara M.","family":"King","sequence":"additional","affiliation":[{"name":"Lifelong Health Theme, SAHMRI, Adelaide 5001 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adeline A.","family":"Lau","sequence":"additional","affiliation":[{"name":"Lifelong Health Theme, SAHMRI, Adelaide 5001 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul J.","family":"Trim","sequence":"additional","affiliation":[{"name":"Proteomics, Metabolomics and MS-Imaging Facility, SAHMRI, Adelaide 5001 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marten F.","family":"Snel","sequence":"additional","affiliation":[{"name":"Proteomics, Metabolomics and MS-Imaging Facility, SAHMRI, Adelaide 5001 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kim M.","family":"Hemsley","sequence":"additional","affiliation":[{"name":"Lifelong Health Theme, SAHMRI, Adelaide 5001 Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"4415","reference":[{"key":"1","doi-asserted-by":"crossref","unstructured":"J Alroy  and JA Lyons  \"J Inborn Errors Metab Screen.\"  \"Lysosomal storage diseases.\" (2014)","DOI":"10.1177\/2326409813517663"},{"key":"2","unstructured":"\"Annual report.\"  (2020)"},{"key":"3","doi-asserted-by":"crossref","unstructured":"EL Aronovich, JM Johnston, P Wang, et al.  \"Genomics.\"  74, \"Molecular basis of mucopolysaccharidosis IIIB in Emu (Dromaius novaehollandiae): An avian model of Sanfilippo syndrome type B.\" 299 (2001)","DOI":"10.1006\/geno.2001.6552"},{"key":"4","doi-asserted-by":"crossref","unstructured":"JR Beggs  and PR Wilson  \"Biol Conserv.\"  56, \"The kaka Nestor meridionalis, a New Zealand parrot endangered by introduced wasps and mammals.\" 23 (1991)","DOI":"10.1016\/0006-3207(91)90086-O"},{"key":"5","doi-asserted-by":"crossref","unstructured":"LM Bergner, IG Jamieson, BC Robertson,  \"Conserv Genet.\"  15, \"Combining genetic data to identify relatedness among founders in a genetically depauperate parrot, the Kakapo (Strigops habroptilus).\" 1013 (2014)","DOI":"10.1007\/s10592-014-0595-y"},{"key":"6","doi-asserted-by":"crossref","unstructured":"D Cook, DR Gardner, JA Pfister,  \"J Agric Food Chem.\"  62, \"Swainsonine-containing plants and their relationship to endophytic fungi.\" 7326 (2014)","DOI":"10.1021\/jf501674r"},{"key":"7","doi-asserted-by":"crossref","unstructured":"N Dussex, J Sainsbury, R Moorhouse, et al.  \"J Hered.\"  106, \"Evidence for Bergman's Rule and not allotropic subspeciation in the threatened kaka (Nestor meridionalis).\" 679 (2015)","DOI":"10.1093\/jhered\/esv079"},{"key":"8","doi-asserted-by":"crossref","unstructured":"N Dussex, D Wegmann, BC Robertson,  \"Mol Ecol.\"  23, \"Postglacial expansion and not human influence best explains the population structure in the endangered kea (Nestor notabilis).\" 2193 (2014)","DOI":"10.1111\/mec.12729"},{"key":"9","doi-asserted-by":"crossref","unstructured":"EE Evans, MP Jones, AJ Crews, et al.  \"J Avian Med Surg.\"  26, \"Neuronal ceroid lipofuscinosis in a Mallard Duck (Anas platyrhynchos).\" 22 (2012)","DOI":"10.1647\/2010-045.1"},{"key":"10","unstructured":"TC Greene  and JR Fraser  \"N Z J Ecol.\"  22, \"Sex ratio of North Island Kaka (Nestor meridionalis septentrionalis), Waihaha Ecological Area, Pureora Forest Park.\" 11 (1998)"},{"key":"11","unstructured":"OK Greiner-Tollersrud  and T Berg  \"Madam Curie bioscience database.\"  \"Lysosomal storage disorders.\" (2013)"},{"key":"12","doi-asserted-by":"crossref","unstructured":"QQ He, PJ Trim, AA Lau, et al.  \"ACS Chem Neurosci.\"  10, \"Synthetic disaccharide standards enable quantitative analysis of stored heparan sulfate in MPS IIIA murine brain regions.\" 3847 (2019)","DOI":"10.1021\/acschemneuro.9b00328"},{"key":"13","doi-asserted-by":"crossref","unstructured":"M Hocquemiller, KM Hemsley, ML Douglass, et al.  \"Mol Ther Methods Clin Dev.\"  17, \"AAVrh10 vector corrects disease pathology in MPS IIIA mice and achieves widespread distribution of SGSH in large animal brains.\" 174 (2020)","DOI":"10.1016\/j.omtm.2019.12.001"},{"key":"14","doi-asserted-by":"crossref","unstructured":"JJ Hopwood, AC Crawley, RM Taylor,  \"Lysosomal diseases of the brain.\"  \"Spontaneous and engineered mammalian models.\" 257 (2004)","DOI":"10.1093\/acprof:oso\/9780198508786.003.0011"},{"key":"15","unstructured":"SA Hunter, AKG Argandona, MR Alley, et al.  \"Kokako.\"  24, \"A lysosomal storage disease in a South Island kaka, Nestor meridionalis.\" 46 (2017)"},{"key":"16","doi-asserted-by":"crossref","unstructured":"IG Jamieson, SS Taylor, LN Tracy, et al.  \"Behav Ecol.\"  20, \"Why some birds do not avoid inbreeding: Insights from New Zealand robins and saddlebacks.\" 575 (2009)","DOI":"10.1093\/beheco\/arp034"},{"key":"17","doi-asserted-by":"crossref","unstructured":"RD Jolly, KE Dittmer, HT Blair,  \"N Z Vet J.\"  64, \"Animal medical genetics: A perspective on the epidemiology and control of inherited diseases.\" 135 (2016)","DOI":"10.1080\/00480169.2015.1129296"},{"key":"18","doi-asserted-by":"crossref","unstructured":"RD Jolly, FI Hill, JA Hill, et al.  \"N Z Vet J.\"  50, \"Polyglucosan body disease in a mixed-breed dog.\" 32 (2002)","DOI":"10.1080\/00480169.2002.36247"},{"key":"19","doi-asserted-by":"crossref","unstructured":"DY Kim, DY Cho, HW Taylor,  \"Vet Pathol.\"  33, \"Lysosomal storage disease in an emu (Dromaius novaehollandiae).\" 365 (1996)","DOI":"10.1177\/030098589603300320"},{"key":"20","doi-asserted-by":"crossref","unstructured":"R Kunita, O Nakabayashi, JY Wu, et al.  \"Biochim Biophys Acta.\"  1362, \"Molecular cloning of acid \u03b1-glucosidase cDNA of Japanese quail (Coturnix coturnix japonica) and the lack of its RNA in acid maltase deficient quails.\" 269 (1998)","DOI":"10.1016\/S0925-4439(97)00092-6"},{"key":"21","doi-asserted-by":"crossref","unstructured":"WC Lamanna, R Lawrence, S Sarrazin, et al.  \"J Biol Chem.\"  286, \"Secondary storage of dermatan sulfate in Sanfilippo disease.\" 6955 (2011)","DOI":"10.1074\/jbc.M110.192062"},{"key":"22","doi-asserted-by":"crossref","unstructured":"J Marsh  and AH Fensom  \"Clin Genet.\"  27, \"4-Methyllumbelliferyl \u03b1-N-acetylylglucosaminidase activity for diagnosis of Sanfilippo B disease.\" 258 (1985)","DOI":"10.1111\/j.1399-0004.1985.tb00217.x"},{"key":"23","doi-asserted-by":"crossref","unstructured":"T Matsui, S Kuroda, M Mizutani, et al.  \"Vet Pathol.\"  29, \"Generalised glycogen storage disease in Japanese quail (Coturnix coturnix japonica).\" 312 (1983)","DOI":"10.1177\/030098588302000307"},{"key":"24","unstructured":"\"Lysosomal storage disorders: A practical guide.\"  197 (2012)"},{"key":"25","doi-asserted-by":"crossref","unstructured":"EH Parlato, DP Armstrong, JG Innes,  \"Oceologia.\"  179, \"Traits influencing range contraction in New Zealand's endemic forest birds.\" 319 (2015)","DOI":"10.1007\/s00442-015-3330-6"},{"key":"26","doi-asserted-by":"crossref","unstructured":"FM Platt, A d'Azzo, BL Davidson, et al.  \"Nat Rev Prim.\"  4, \"Lysosomal storage diseases.\" 27 (2018)","DOI":"10.1038\/s41572-018-0025-4"},{"key":"27","unstructured":"JS Proudfoot, MM Garner, D Prieur, et al.  \"Proceedings of the American Association of Zoo Veterinarians annual conference international, Association for Aquatic Animal Medicine, New Orleans, Louisiana, 17\u201321 September.\"  \"Lysosomal storage disease in Costa's hummingbirds (Calypte costae).\" 305 (2000)"},{"key":"28","doi-asserted-by":"crossref","unstructured":"RL Reece  and P MacWhirter  \"Vet Rec.\"  122, \"Neuronal ceroid-lipofuscinosis in a lovebird.\" 187 (1988)","DOI":"10.1136\/vr.122.8.187"},{"key":"29","doi-asserted-by":"crossref","unstructured":"PJ Trim, JJ Hopwood, MF Snel,  \"Anal Chem.\"  87, \"Butanolysis derivatization: Improved sensitivity in LC-MS\/MS quantitation of heparan sulfate in urine from mucopolysaccharidosis patients.\" 9243 (2015)","DOI":"10.1021\/acs.analchem.5b01743"},{"key":"30","doi-asserted-by":"crossref","unstructured":"PJ Trim, AA Lau, JJ Hopwood, et al.  \"Rapid Commun Mass Spectrom.\"  28, \"A simple method for early age phenotype confirmation using toe tissue from a mouse model of MPS IIIA.\" 933 (2014)","DOI":"10.1002\/rcm.6861"},{"key":"31","doi-asserted-by":"crossref","unstructured":"YV Voznyi, JL Keulemans, OP van Diggelen,  \"J Inherit Metabol Dis.\"  24, \"A fluorimetric enzyme assay for the diagnosis of MPS II (Hunter disease).\" 675 (2001)","DOI":"10.1023\/A:1012763026526"},{"key":"32","doi-asserted-by":"crossref","unstructured":"PAL. Wight,  \"Neuropathol Appl Neurobiol.\"  2, \"The histopathology of a cerebral lipidosis in the Hawaiian goose, Branta sandvicensis.\" 335 (1976)","DOI":"10.1111\/j.1365-2990.1976.tb00508.x"},{"key":"33","doi-asserted-by":"crossref","unstructured":"MJ Wilkerson, DC Lewis, SL Marks, et al.  \"Vet Pathol.\"  35, \"Clinical and morphological features of mucopolysaccharidosis type II in a dog: Naturally occurring model of Hunter syndrome.\" 230 (1998)","DOI":"10.1177\/030098589803500311"},{"key":"34","doi-asserted-by":"crossref","unstructured":"A W\u00fcnschmann, A Armien, R Wallace, et al.  \"Vet Pathol.\"  43, \"Neuronal storage disease in a group of captive Humboldt penguins (Spheniscus humboldti).\" 1029 (2006)","DOI":"10.1354\/vp.43-6-1029"},{"key":"35","doi-asserted-by":"crossref","unstructured":"BJ Zeng, PA Torres, TC Viner, et al.  \"Mol Genet Metab.\"  95, \"Spontaneous appearance of Tay-Sachs disease in an animal model.\" 59 (2008)","DOI":"10.1016\/j.ymgme.2008.06.010"}],"container-title":["Journal of Wildlife Diseases"],"original-title":[],"deposited":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T08:28:35Z","timestamp":1793435315000},"score":1,"resource":{"primary":{"URL":"https:\/\/bioone.org\/journals\/journal-of-wildlife-diseases\/volume-57\/issue-4\/JWD-D-20-00173\/MUCOPOLYSACCHARIDOSIS-II-MPS-II-IN-A-FREE-LIVING-KAKA-NESTOR\/10.7589\/JWD-D-20-00173.full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,11]]},"references-count":35,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2021,10,1]]}},"URL":"https:\/\/doi.org\/10.7589\/jwd-d-20-00173","relation":{},"ISSN":["0090-3558","1943-3700"],"issn-type":[{"value":"0090-3558","type":"print"},{"value":"1943-3700","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,10,11]]}}}