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Sheep health
GM2 gangliosidosis in British Jacob sheep
  1. Mark Wessels
  1. AHVLA – Preston, Barton Hall, Garstang Road, Barton, Preston, Lancashire PR3 5HE
  1. Helen Carty
  1. Ayr Disease Surveillance Centre, Auchincruive, Ayr KA6 5AE
  1. Pauline Baird and
  2. Norman Beggs
  1. Agri-Food and Biosciences Institute, 43 Beltany Road, Coneywarren, Omagh, County Tyrone BT78 5NF
  1. Timothy Cox,
  2. Begoña Cachón and
  3. Susan Wang
  1. Department of Medicine, University of Cambridge, Addenbrooke's Hospital, Cambridge CB2 0QQ
  1. Ian Davies and
  2. Paul Holmes
  1. AHVLA – Shrewsbury, Kendal Road, Harlescott, Shrewsbury SY1 4HD
  1. Adrienne Mackintosh
  1. AHVLA – Carmarthen, Jobs Well Road, Johnstown, Carmarthen SA31 3EZ
  1. Francesca Chianini
  1. Moredun Research Institute, Pentlands Science Park, Bush Loan, Midlothian EH26 0PZ
  1. e-mail: mark.wessels{at}ahvla.gsi.gov.uk

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GM2 gangliosidosis (Tay-Sachs disease) is a prototypical lysosomal storage disease first described in people in the 19th century. Defects in the ß-hexosaminidase A enzyme lead to the accumulation of GM2 ganglioside within lysosomes, particularly within the central nervous system. The disease is characterised by widespread and relentless neurodegeneration with variable onset and severity related to the degree of enzyme deficiency.

Naturally occurring GM2 gangliosidosis has been described in several species of domesticated and wild animals and has recently been reported in Jacob sheep from North America (Torres and others 2010, Porter and others …

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