Addenda page on inside back cover. / Bibliography: leaves 153-176. / xiii, 176 leaves ; ill. (some col.) ; 30 cm / Title page, contents and abstract only. The complete thesis in print form is available from the University Library. / Mucopolysaccharideosis type IIIA (MPS IIIA, Sanfilippo A syndrome) is an autosomal recessive lysosomal storage disease, with a prevalence in Australia of 1 in 114,000. MPS IIIA is caused by a deficiency of the lysosomal enzyme sulphamidase which is needed together with other exohydrolases and a N-acetyltransferase to break down the glycosaminoglycan heparan sulphate to sulphate and monosaccharides. Patients are characterised by severe central nervous systems degeneration together with mild somatic involvement; this disproportionate correlation is unique amongst the mucopolysaccharidoses. Features include severe behavioural disturbances, such as hyperactivity and aggressiveness, coarse hair and mild hepatosplenomegaly. Death is usually in the mid- to late-teenage years. Enzyme replacement therapy by intravenous administration of recombinant human NS (rhNS) has been proposed as a potential therapy for MPS IIIA. This thesis suggests that rhNS, entering the brain in the first few weeks of life, is able to retard the behaviour and learning difficulties in MPS IIIA mice. / Thesis (Ph.D.)--University of Adelaide, Dept. of Paediatrics, 2003

Thesis (Ph.D.)--University of Hong Kong, 1994. / Includes bibliographical references. Also available in print.

Thesis (M.A.)--State University of New York at Binghamton, Psychology Department, 2006. / Includes bibliographical references.

Thesis (Ph. D.)--State University of New York at Binghamton, Psychology Department, 2006. / Includes bibliographical references.

Thesis (Ph.D.)--University of Hong Kong, 1994. / Includes bibliographical references. Also available in print.

Thesis (Ph. D.)--University of Missouri--Columbia, 2000. / "December 2000." Typescript. Vita. Includes bibliographical references (leaves 95-112). Also available on the Internet.

Thesis (M. Phil.)--University of Hong Kong, 2009. / Includes bibliographical references (leaves 164-173). Also available online.

Thesis (Ph. D.)--University of Texas at Austin, 2000. / Vita. Includes bibliographical references (leaves 112-125). Available also in a digital version from Dissertation Abstracts.

Thesis (M.S.)--University of Massachusetts Amherst, 2009. / Includes bibliographical references (p. 47-59).

Thesis (Ph. D.)--University of Hong Kong, 2010. / Includes bibliographical references (p. 256-292). Also available in print.