DOI: 10.1083/jcb.200012098. The BPAG1 locus

The BPAG1 locus

10.1083/jcb.200012098

Crossref journal-article

Rockefeller University Press

The Journal of Cell Biology (291)

Abstract

Bullous pemphigoid antigen 1 (BPAG1) is a member of the plakin family with cytoskeletal linker properties. Mutations in BPAG1 cause sensory neuron degeneration and skin fragility in mice. We have analyzed the BPAG1 locus in detail and found that it encodes different interaction domains that are combined in tissue-specific manners. These domains include an actin-binding domain (ABD), a plakin domain, a coiled coil (CC) rod domain, two different potential intermediate filament–binding domains (IFBDs), a spectrin repeat (SR)-containing rod domain, and a microtubule-binding domain (MTBD). There are at least three major forms of BPAG1: BPAG1-e (302 kD), BPAG1-a (615 kD), and BPAG1-b (834 kD). BPAG1-e has been described previously and consists of the plakin domain, the CC rod domain, and the first IFBD. It is the primary epidermal BPAG1 isoform, and its absence that is the likely cause of skin fragility in mutant mice. BPAG1-a is the major isoform in the nervous system and a homologue of the microtubule actin cross-linking factor, MACF. BPAG1-a is composed of the ABD, the plakin domain, the SR-containing rod domain, and the MTBD. The absence of BPAG1-a is the likely cause of sensory neurodegeneration in mutant mice. BPAG1-b is highly expressed in muscles, and has extra exons encoding a second IFBD between the plakin and SR-containing rod domains of BPAG1-a.

Bibliography

Leung, C. L., Zheng, M., Prater, S. M., & Liem, R. K. H. (2001). The BPAG1 locus. The Journal of Cell Biology, 154(4), 691â698.

Authors 4

Conrad L. Leung (first)
Min Zheng (additional)
Susan M. Prater (additional)
Ronald K.H. Liem (additional)

References 18 Referenced 158

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Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 12:46 p.m.)
Deposited	2 years, 1 month ago (July 29, 2023, 12:30 a.m.)
Indexed	2 months ago (July 2, 2025, 2:31 p.m.)
Issued	24 years ago (Aug. 20, 2001)
Published	24 years ago (Aug. 20, 2001)
Published Online	24 years ago (Aug. 20, 2001)
Published Print	24 years ago (Aug. 20, 2001)

Funders 0

None

BibTeX

@article{Leung_2001, title={The BPAG1 locus}, volume={154}, ISSN={0021-9525}, url={http://dx.doi.org/10.1083/jcb.200012098}, DOI={10.1083/jcb.200012098}, number={4}, journal={The Journal of Cell Biology}, publisher={Rockefeller University Press}, author={Leung, Conrad L. and Zheng, Min and Prater, Susan M. and Liem, Ronald K.H.}, year={2001}, month=aug, pages={691–698} }

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