Delivery of neurturin by AAV2 (CERE-120)-mediated gene transfer provides structural and functional neuroprotection and neurorestoration in MPTP-treated monkeys

Ann Neurol. 2006 Dec;60(6):706-15. doi: 10.1002/ana.21032.

Abstract

Objective: We tested the hypothesis that gene delivery of the trophic factor neurturin could preserve motor function and protect nigrostriatal circuitry in hemiparkinsonian monkeys.

Methods: An adeno-associated virus-based vector encoding human neurturin (AAV2-NTN; also called CERE-120) was injected into the striatum and substantia nigra of monkeys 4 days after a unilateral intracarotid injection of N-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) rendered them hemiparkinsonian. Control hemiparkinsonian monkeys received either AAV2 encoding green fluorescent protein or formulation buffer.

Results: Although stable deficits were seen in all control monkeys, AAV2-NTN significantly improved MPTP-induced motor impairments by 80 to 90% starting at approximately month 4 and lasting until the end of the experiment (month 10). AAV2-NTN significantly preserved nigral neurons, significantly preserved striatal dopaminergic innervation, and activated phospho-extracellular signal-regulated kinase, consistent with a mechanism involving a trophic factor-initiated molecular cascade. Histological analyses of numerous brain regions, including the cerebellum, showed normal cytoarchitecture and no aberrant pathology.

Interpretation: These data demonstrate that AAV2-NTN (CERE-120) can preserve function and anatomy in degenerating nigrostriatal neurons and are supportive of ongoing clinical tests in Parkinson's disease patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Corpus Striatum / metabolism
  • Corpus Striatum / pathology
  • Corpus Striatum / physiopathology
  • Cytoprotection / genetics
  • Disease Models, Animal
  • Dopamine / metabolism
  • Extracellular Signal-Regulated MAP Kinases / metabolism
  • Gene Transfer Techniques
  • Genetic Therapy / methods*
  • Genetic Vectors / genetics
  • Genetic Vectors / metabolism
  • Genetic Vectors / therapeutic use*
  • Macaca mulatta
  • Male
  • Nerve Regeneration / genetics
  • Neural Pathways / metabolism
  • Neural Pathways / pathology
  • Neural Pathways / physiopathology
  • Neurons / metabolism
  • Neurons / pathology
  • Neurturin / genetics*
  • Neurturin / metabolism
  • Parkinsonian Disorders / genetics
  • Parkinsonian Disorders / metabolism
  • Parkinsonian Disorders / therapy*
  • Recovery of Function / genetics
  • Substantia Nigra / metabolism
  • Substantia Nigra / pathology
  • Substantia Nigra / physiopathology
  • Treatment Outcome

Substances

  • Neurturin
  • Extracellular Signal-Regulated MAP Kinases
  • Dopamine