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Updated: Feb 28, 2026

Targeting Alpha Synuclein Aggregates in Cutaneous Peripheral Nerve Fibers by Free-floating Immunofluorescence Assay
Published on: June 25, 2019
BPAG1, a distinctive role in skin and neurological diseases
Arshad Ali1, Lifang Hu1, Fan Zhao1
1Laboratory for Bone Metabolism, Key Laboratory for Space Bioscience and Biotechnology, School of Life Sciences, Northwestern Polytechnical University, Xi'an, Shaanxi 710072, PR China; Shenzhen Research Institution of Northwestern Polytechnical University, Shenzhen, 518057, PR China; Northwestern Polytechnical University-Hong Kong Baptist University Joint Research Centre for Translational Medicine on Musculoskeletal Health in Space, Xi'an, 710072, PR China.
Abstract:
Spectraplakins are multifunctional cytoskeletal linker proteins that act as important communicators, connecting cytoskeletal components with each other and to cellular junctions. Bullous pemphigoid antigen 1 (BPAG1)/dystonin is a member of spectraplakin family and expressed in various tissues. Alternative splicing of BPAG1 gene produces various isoforms with unique structure and domains. BPAG1 plays crucial roles in numerous biological processes, such as cytoskeleton organization, cell polarization, cell adhesion, and cell migration as well as signaling transduction. Genetic mutation of BPAG1 isoforms is the miscreant of epidermolysis bullosa and multifarious, destructive neurological diseases. In this review, we summarize the recent advances of BPAG1's role in various biological processes and in skin and neurological diseases.
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