Play all audios:
ABSTRACT INTERMEDIATE FILAMENTS REQUIRE SMALL HEAT SHOCK PROTEIN CHAPERONES TO MAINTAIN THEIR STRUCTURAL INTEGRITY AND PREVENT THEIR AGGREGATION; MUTATIONS IN THESE PROTEINS LEAD TO MYOPATHY
AND CATARACT. Access through your institution Buy or subscribe This is a preview of subscription content, access via your institution RELEVANT ARTICLES Open Access articles citing this
article. * ΑB-CRYSTALLIN IS A SENSOR FOR ASSEMBLY INTERMEDIATES AND FOR THE SUBUNIT TOPOLOGY OF DESMIN INTERMEDIATE FILAMENTS * Sarika Sharma * , Gloria M. Conover * … Roy A. Quinlan _Cell
Stress and Chaperones_ Open Access 03 May 2017 ACCESS OPTIONS Access through your institution Subscribe to this journal Receive 12 print issues and online access $209.00 per year only $17.42
per issue Learn more Buy this article * Purchase on SpringerLink * Instant access to full article PDF Buy now Prices may be subject to local taxes which are calculated during checkout
ADDITIONAL ACCESS OPTIONS: * Log in * Learn about institutional subscriptions * Read our FAQs * Contact customer support REFERENCES * Goldfarb, L.G. et al. Missense mutations in desmin
associated with familial cardiac and skeletal myopathy. _Nature Genet._ 19, 402–403 (1998). Article CAS Google Scholar * Fuchs, E. & Cleveland, D.W. A structural scaffolding of
intermediate filaments in health and disease. _Science_ 279, 514–519 (1998). Article CAS Google Scholar * Iwaki, T., Kume-Iwaki, A., Liem, R.K.H. & Goldman, J.E. aB-crystallin is
expressed in non-lenticular tissues and accumulates in Alexander's disease brain. _Cell_ 57, 71–78 (1989). Article CAS Google Scholar * Vicart, P. et al. A missense mutation in the
aB-crystallin chaperone gene causes a desmin-related myopathy. _Nature Genet._ 20, 92–95 (1998). Article CAS Google Scholar * Muñoz-Mármol, A.M. et al. A dysfunctional desmin mutation in
a patient with severe generalized myopathy. _Proc. Natl. Acad. Sci. USA_, 95, 11312–11317 (1998). Article Google Scholar * Nicholl, I.D. & Quinlan, R.A. Chaperone activity of
a-crystallins modulates intermediate filament assembly. _EMBO J._ 13, 945–953 (1994). Article CAS Google Scholar * Carter, J.M., Hutcheson, A.M. & Quinlan, R.A. _In vitro_ studies on
the assembly properties of the lens proteins CP49, CP115: Co-assembly with a-crystallin but not with vimentin. _Exp. Eye Res._ 60, 181–192 (1995). Article CAS Google Scholar * Litt, M. et
al. Autosomal dominant mutation of congenital cataract associated with a missense mutation in the human a-crystallin gene CRYAA. _Hum. Mol. Genet._ 7, 471–474 (1998). Article CAS Google
Scholar * Kim, K.K., Kim, R. & Kim, S.H. Crystal structure of a small heat-shock protein. _Nature_ 394, 595–599 (1998). Article CAS Google Scholar * Boelens, W.C., Croes, Y., de
Ruwe, M., de Reu, L. & de Jong, W.W. Negative charges in the C-terminal domain stabilize the αB-crystallin complex. _J. Biol. Chem._ 273, 28055–28090 (1998). Article Google Scholar *
Muchowski, P.J., Valdez, M.M. & Clark, J.I. αB-crystallin selectively targets intermediate filament proteins during thermal stress. _Invest. Ophthalmol. Vis. Sci_. 40, in the press.
Download references AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Biochemistry, MSI/WTB Complex, University of Dundee, Dow Street, Dundee, DD1 5EH, Scotland Roy Quinlan &
Paul van den IJssel Authors * Roy Quinlan View author publications You can also search for this author inPubMed Google Scholar * Paul van den IJssel View author publications You can also
search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Quinlan, R., van den IJssel, P. Fatal attraction: When
chaperone turns harlot. _Nat Med_ 5, 25–26 (1999). https://doi.org/10.1038/4704 Download citation * Issue Date: January 1999 * DOI: https://doi.org/10.1038/4704 SHARE THIS ARTICLE Anyone you
share the following link with will be able to read this content: Get shareable link Sorry, a shareable link is not currently available for this article. Copy to clipboard Provided by the
Springer Nature SharedIt content-sharing initiative