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pro vyhledávání: '"Katia J. Evans"'
Autor:
Christian W. Keller, Bryan Conn, Brett Lauring, Kristen Glasgow, Karen S. Pavur, Katia J. Evans
Publikováno v:
Proceedings of the National Academy of Sciences. 103:10666-10671
Hereditary spastic paraplegia (HSP) is a neurodegenerative disorder that is characterized by retrograde axonal degeneration that primarily affects long spinal neurons. The disease is clinically heterogeneous, and there are >20 genetic loci identified
Publikováno v:
The Journal of Cell Biology
Mutations in the AAA adenosine triphosphatase (ATPase) Spastin (SPG4) cause an autosomal dominant form of hereditary spastic paraplegia, which is a retrograde axonopathy primarily characterized pathologically by the degeneration of long spinal neuron
Publikováno v:
The Journal of Cell Biology
Spastin, an AAA ATPase mutated in the neurodegenerative disease hereditary spastic paraplegia, severs microtubules. Many other AAA proteins form ring-shaped hexamers and contain pore loops, which project into the ring's central cavity and act as ratc