An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments

Cell. 1996 Aug 23;86(4):655-65. doi: 10.1016/s0092-8674(00)80138-5.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Actin Cytoskeleton / chemistry*
  • Alternative Splicing
  • Animals
  • Autoantigens / physiology*
  • Brain Chemistry
  • Carrier Proteins*
  • Collagen*
  • Cytoskeletal Proteins*
  • Dystonin
  • Fluorescent Antibody Technique
  • Ganglia, Spinal / ultrastructure
  • Gene Expression
  • Humans
  • In Situ Hybridization
  • Intermediate Filaments / chemistry*
  • Mice
  • Mice, Mutant Strains
  • Microfilament Proteins / physiology*
  • Molecular Weight
  • Nerve Tissue Proteins / chemistry*
  • Neurofilament Proteins / metabolism*
  • Neurons, Afferent / ultrastructure*
  • Non-Fibrillar Collagens*
  • Protein Binding
  • RNA, Messenger / genetics
  • Spinal Cord / chemistry


  • Autoantigens
  • Carrier Proteins
  • Cytoskeletal Proteins
  • DST protein, human
  • Dst protein, mouse
  • Dystonin
  • Microfilament Proteins
  • Nerve Tissue Proteins
  • Neurofilament Proteins
  • Non-Fibrillar Collagens
  • RNA, Messenger
  • collagen type XVII
  • Collagen