Mucopolysaccharidosis II (MPS II) in a free-living kaka (nestor meridionalis) in New Zealand

Robert D. Jolly, Stuart A. Hunter, Maurice R. Alley, Barbara King, Adeline Bowen, Paul J. Trim, Marten F. Snel, Kim Hemsley

Research output: Contribution to journalArticlepeer-review

1 Citation (Scopus)


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.

Original languageEnglish
Pages (from-to)884-890
Number of pages7
JournalJournal of Wildlife Diseases
Issue number4
Early online date23 Aug 2021
Publication statusPublished or Issued - 2021


  • Enzymology
  • Hunter syndrome
  • Lysosomal storage disease
  • Mass spectrometry
  • Mucopolysaccharidosis II
  • Parrot
  • Ultrastructure

ASJC Scopus subject areas

  • Ecology, Evolution, Behavior and Systematics
  • Ecology

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