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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments.

Typified by rapid degeneration of sensory neurons, dystonia musculorum mice have a defective BPAG1 gene, known to be expressed in epidermis. We report a neuronal splice form, BPAG1n, which localizes to sensory axons. Both isoforms have a coiled-coil rod, followed by a carboxy domain that associates with intermediate filaments. However, the amino terminus of BPAG1n differs from BPAG1e in that it contains a functional actin-binding domain. In transfected cells, BPAG1n coaligns neurofilaments and microfilaments, establishing this as a cytoskeletal protein interconnecting actin and intermediate filament cytoskeletons. In BPAG1 null mice, axonal architecture is markedly perturbed, consistent with a failure to tether neurofilaments to the actin cytoskeleton and underscoring the physiological relevance of this protein.[1]

References

  1. An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments. Yang, Y., Dowling, J., Yu, Q.C., Kouklis, P., Cleveland, D.W., Fuchs, E. Cell (1996) [Pubmed]
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