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Third-party-funded project

Identification of the gene defect in a mouse mutant "progressive motor neuronopathy"

Project management at the University of Würzburg:

Participating scientists:

The autosomal recessive mouse mutant pmn has been identified 14 years ago and since then used as an animal model for motoneuron disease. We have identified the underlying gene defect by positional cloning. The mutation, a single nucleotide exchange in the Tbce gene leads to a single amino-acid exchange in CofE, an essential component of the chaperone machinery for assembly of microtubuli. This defect is responsible for axonal swellings and reduced axonal outgrowth.

Key words:
    Motoneuron disease
    Amyotrophic lateral sclerosis
    Tubuline assembly

Projekt period: from 01.1996 to 12.2004

Funding institution:
DFG ( SFB 581 TP V1 ) ,Granting date: 18.08.1998
Sonstige Stiftung ( Schilling-Stiftung )
Bund ( BMBF-Verbund-Projekt "Verbesserung der axonalen Regeneration nach Nervenverletzungen mit einem optimierten in-vitro System" )

Preceding project:
DFG to 61/8-4


Institute for Clinical Neurobiology