Slowed progression in models of Huntington disease by adipose stem cell transplantation

Ann Neurol. 2009 Nov;66(5):671-81. doi: 10.1002/ana.21788.

Abstract

Objective: Adipose-derived stem cells (ASCs) are readily accessible and secrete multiple growth factors. Here, we show that ASC transplantation rescues the striatal pathology of Huntington disease (HD) models.

Methods: ASCs were isolated from human subcutaneous adipose tissue. In a quinolinic acid (QA)-induced rat model of striatal degeneration, human ASCs (1 million cells) were transplanted into the ipsilateral striatal border immediately after the QA injection. In 60-day-old R6/2 mice transgenic for HD, ASCs (0.5 million cells) were transplanted into each bilateral striata. In in vitro experiments, we treated mutant huntingtin gene-transfected cerebral neurons with ASC-conditioned media.

Results: In the QA model, human ASCs reduced apomorphine-induced rotation behavior, lesion volume, and striatal apoptosis. In R6/2 transgenic mice, transplantation of ASCs improved Rota-Rod performance and limb clasping, increased survival, attenuated the loss of striatal neurons, and reduced the huntingtin aggregates. ASC-transplanted R6/2 mice expressed elevated levels of peroxisome proliferator-activated receptor gamma coactivator-1alpha (PGC-1alpha) and reactive oxygen defense enzymes and showed activation of the Akt/cAMP-response element-binding proteins. ASC-conditioned media decreased the level of N-terminal fragments of mutant huntingtin and associated apoptosis, and increased PGC-1alpha expression.

Interpretation: Collectively, ASC transplantation slowed striatal degeneration and behavioral deterioration of HD models, possibly via secreted factors.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adipocytes / transplantation*
  • Animals
  • Cell Line, Tumor
  • Cells, Cultured
  • Corpus Striatum / metabolism
  • Corpus Striatum / pathology
  • Culture Media, Conditioned
  • Disease Models, Animal*
  • Disease Progression
  • Humans
  • Huntington Disease / chemically induced
  • Huntington Disease / pathology
  • Huntington Disease / prevention & control*
  • Huntington Disease / surgery*
  • Male
  • Mice
  • Mice, Transgenic
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / pathology
  • Quinolinic Acid / administration & dosage
  • Rats
  • Reactive Oxygen Species / antagonists & inhibitors
  • Stem Cell Transplantation* / methods

Substances

  • Culture Media, Conditioned
  • Reactive Oxygen Species
  • Quinolinic Acid