Outcome of cell suspension allografts in a patient with Huntington's disease.

Abstract

For patients with incurable neurodegenerative disorders such as Huntington's (HD) and Parkinson's disease, cell transplantation has been explored as a potential treatment option. Here, we present the first clinicopathological study of a patient with HD in receipt of cell-suspension striatal allografts who took part in the NEST-UK multicenter clinical transplantation trial. Using various immunohistochemical techniques, we found a discrepancy in the survival of grafted projection neurons with respect to grafted interneurons as well as major ongoing inflammatory and immune responses to the grafted tissue with evidence of mutant huntingtin aggregates within the transplant area. Our results indicate that grafts can survive more than a decade post-transplantation, but show compromised survival with inflammation and mutant protein being observed within the transplant site. Ann Neurol 2018;84:950-956.

• Authors: Alexander Maxan, Sarah Mason, Martine Saint-Pierre, Emma Smith, Aileen Ho, Timothy Harrower, Colin Watts, Yen Tai, Nicola Pavese, Julie C Savage, Marie-Ève Tremblay, Peter Gould, Anne E Rosser, Stephen B Dunnett, Paola Piccini, Roger A Barker, Francesca Cicchetti
• Journal: Annals of neurology (Ann Neurol) Vol. 84 Issue 6 Pg. 950-956 (12 2018) ISSN: 1531-8249 [Electronic] United States
• PMID: 30286516 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2018 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.

Chemical References

• Antigens, CD
• Calbindin 2
• HTT protein, human
• Huntingtin Protein
• Nerve Tissue Proteins
• Parvalbumins
• Acetylcholinesterase

Topics

• Acetylcholinesterase (metabolism)
• Adult
• Allografts (pathology)
• Antigens, CD (metabolism)
• Brain (pathology)
• Brain Tissue Transplantation (methods)
• Calbindin 2 (metabolism)
• Humans
• Huntingtin Protein (genetics)
• Huntington Disease (genetics, surgery)
• Interneurons (metabolism, pathology)
• Male
• Microglia (metabolism, pathology)
• Nerve Tissue Proteins (metabolism)
• Parvalbumins (metabolism)