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Emerging therapies for neurodegenerative lysosomal storage disorders - from concept to reality

  • Review
  • Published:
Journal of Inherited Metabolic Disease

Abstract

Lysosomal storage disorders are inherited metabolic diseases in which a mutation in a gene encoding a lysosomal enzyme or lysosome-related protein results in the intra-cellular accumulation of substrate and reduced cell/tissue function. Few patients with neurodegenerative lysosomal storage disorders have access to safe and effective treatments although many therapeutic strategies have been or are presently being studied in vivo thanks to the availability of a large number of animal models. This review will describe the comparative advancement of a variety of therapeutic strategies through the ‘research pipeline’. Our goal is to provide information for clinicians, researchers and patients/families alike on the leading therapeutic candidates at this point in time, and also to provide information on emerging approaches that may provide a safe and effective treatment in the future. The length of the pipeline represents the significant and sustained effort required to move a novel concept from the laboratory into the clinic.

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Role of funding sources

The authors have received funding for their research from the Australian National Health and Medical Research Council of Australia, the Sanfilippo Children’s Research Foundation (Canada), Swiss Sanfilippo Foundation, Alliance Sanfilippo, the National MPS Society (USA), the Isaac Foundation, the Women’s and Children’s Hospital Research Foundation and Shire Human Genetic Therapies. The funding sources did not have any role in the writing of this review or in the decision to submit it for publication.

Competing interest

International patents are held by JJH and others for mammalian sulphamidase, iduronate 2-sulphatase and α-iduronidase and genetic sequences encoding these proteins for use in the investigation, diagnosis and treatment of subjects suspected of suffering from sulphamidase, iduronate-2-sulphatase and α-iduronidase deficiencies (US Patent # 5863782, 5728381 and 6149909, respectively).

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  1. Lysosomal Diseases Research Unit, 4th Floor Rogerson Building, SA Pathology, Women’s and Children’s Hospital campus, 72 King William Road, North Adelaide, SA, 5006, Australia

    Kim M. Hemsley & John J. Hopwood

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  1. Kim M. Hemsley
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  2. John J. Hopwood
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Correspondence to Kim M. Hemsley.

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Communicated by: Gregory M. Pastores

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Hemsley, K.M., Hopwood, J.J. Emerging therapies for neurodegenerative lysosomal storage disorders - from concept to reality. J Inherit Metab Dis 34, 1003–1012 (2011). https://doi.org/10.1007/s10545-011-9341-5

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