Canine heparan sulfate sulfamidase and the molecular pathology underlying Sanfilippo syndrome type A in Dachshunds.

@article{Aronovich2000CanineHS,
  title={Canine heparan sulfate sulfamidase and the molecular pathology underlying Sanfilippo syndrome type A in Dachshunds.},
  author={Elena L. Aronovich and K. Paige Carmichael and Hiroki Morizono and Ioannis G. Koutlas and Minerva Deanching and George M Hoganson and Alain Fischer and Chester B. Whitley},
  journal={Genomics},
  year={2000},
  volume={68 1},
  pages={
          80-4
        }
}
Heparan sulfate sulfamidase (HSS) is a lysosomal exohydrolase that, when deficient, results in intralysosomal accumulation of heparan sulfate and the clinical phenotype of Sanfilippo syndrome type A. The first animal disease homolog of human Sanfilippo syndrome type A has been recently indentified in Dachshund littermates. To identify the molecular defect, the nucleotide sequences of the normal canine HSS gene and cDNA were determined using PCR-based approaches. The coding region showed 87… CONTINUE READING
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