Zobrazeno 1 - 10
of 13
pro vyhledávání: '"Joanna M. Solowska"'
Publikováno v:
Molecular Biology of the Cell
The SPAST gene, which produces two isoforms of the microtubule-severing protein spastin, is the chief gene mutated in hereditary spastic paraplegia. Truncated M1 spastin proteins are toxic and have the potential to accumulate in these patients.
Autor:
Peter W. Baas, Joanna M. Solowska
Publikováno v:
Brain. 138:2471-2484
Mutations in more than 70 distinct loci and more than 50 mutated gene products have been identified in patients with hereditary spastic paraplegias, a diverse group of neurological disorders characterized predominantly, but not exclusively, by progre
Publikováno v:
Human Molecular Genetics. 19:2767-2779
The spectrum of mutations (missense, non-sense and splice-site) associated with hereditary spastic paraplegia 4 (HSP-SPG4) (SPG4:OMIM#182601) has suggested that this autosomal dominant disease results from loss of function. Because the protein encode
Publikováno v:
Molecular Biology of the Cell
This article demonstrates that the augmentation of axonal branching induced by bFGF is explicable on the basis of an enhancement of microtubule-severing, and that three different proteins related to microtubule-severing are affected by treatment of n
Autor:
Gerardo Morfini, B T Himes, Peter W. Baas, Scott T. Brady, Aditi Falnikar, Joanna M. Solowska, Dongyang Huang
Publikováno v:
The Journal of Neuroscience. 28:2147-2157
Spastin and P60-katanin are two distinct microtubule-severing proteins. Autosomal dominant mutations in the SPG4 locus corresponding to spastin are the most common cause of hereditary spastic paraplegia (HSP), a neurodegenerative disease that afflict
Publikováno v:
The Journal of Neuroscience. 24:5778-5788
Katanin is a heterodimeric enzyme that severs microtubules from the centrosome so that they can move into the axon. Katanin is broadly distributed in the neuron, and therefore presumably also severs microtubules elsewhere. Such severing would generat
Autor:
Joanna M. Solowska, Muriel Chauvière, Wenqian Yu, Agnieszka Mazurek, Peter W. Baas, Douglas H. Baird, Michel Kress, Daniel W. Buster
Publikováno v:
Journal of Neurocytology. 32:79-96
Kif15 is a kinesin-related protein whose mitotic homologues are believed to crosslink and immobilize spindle microtubules. We have obtained rodent sequences of Kif15, and have studied their expression and distribution in the developing nervous system
Publikováno v:
Molecular and Cellular Neuroscience. 21:266-284
To assess the role of semaphorin 3A (Sema3A) and its receptor component neuropilin-1 (Npn-1) in pontocerebellar axon guidance, we compared the distributions of Sema3A, Npn-1, and DiI-labeled pontocerebellar axons in neonatal mouse cerebellum. Between
Autor:
Douglas H. Baird, Joanna M. Solowska, Yuling Luo, Barbara Kruk, Jonathan A. Raper, Sylvia A. Rabacchi
Publikováno v:
The Journal of Neuroscience. 19:4437-4448
Most axons in the CNS innervate specific subregions or layers of their target regions and form contacts with specific types of target neurons, but the molecular basis of this process is not well understood. To determine whether collapsin-1/semaphorin
Autor:
Mitchell D'Rozario, Michael W. Davidson, Daniel R. Marenda, Peter W. Baas, Daphney C. Jean, Joanna M. Solowska
Publikováno v:
The Journal of neuroscience : the official journal of the Society for Neuroscience. 34(5)
Mutations to theSPG4gene encoding the microtubule-severing protein spastin are the most common cause of hereditary spastic paraplegia. Haploinsufficiency, the prevalent model for the disease, cannot readily explain many of its key aspects, such as it