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Science 23 January 1998:
Vol. 279. no. 5350, pp. 514 - 519
DOI: 10.1126/science.279.5350.514
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Articles

A Structural Scaffolding of Intermediate Filaments in Health and Disease

Elaine Fuchs, Don W. Cleveland *

The cytoplasm of animal cells is structured by a scaffolding composed of actin microfilaments, microtubules, and intermediate filaments. Intermediate filaments, so named because their 10-nanometer diameter is intermediate between that of microfilaments (6 nanometers) and microtubules (23 nanometers), assemble into an anastomosed network within the cytoplasm. In combination with a recently identified class of cross-linking proteins that mediate interactions between intermediate filaments and the other cytoskeletal networks, evidence is reviewed here that intermediate filaments provide a flexible intracellular scaffolding whose function is to structure cytoplasm and to resist stresses externally applied to the cell. Mutations that weaken this structural framework increase the risk of cell rupture and cause a variety of human disorders.

E. Fuchs is at the Howard Hughes Medical Institute and Department of Molecular Genetics and Cell Biology, University of Chicago, Chicago, IL 60637, USA. D. W. Cleveland is at the Ludwig Institute for Cancer Research and the Departments of Medicine and Neuroscience, University of California at San Diego, La Jolla, CA 92093, USA.
*   To whom correspondence should be addressed.

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J Appl Physiol 91, 1487-1500
   Abstract »    Full Text »    PDF »
Endothelial Oxidative Stress Activates the Lectin Complement Pathway : Role of Cytokeratin 1.
C. D. Collard, M. C. Montalto, W. R. Reenstra, J. A. Buras, and G. L. Stahl (2001)
Am. J. Pathol. 159, 1045-1054
   Abstract »    Full Text »    PDF »
Simple epithelium keratins 8 and 18 provide resistance to Fas-mediated apoptosis. The protection occurs through a receptor-targeting modulation.
S. Gilbert, A. Loranger, N. Daigle, and N. Marceau (2001)
J. Cell Biol. 154, 763-774
   Abstract »    Full Text »    PDF »
Cutting Edge: Integration of Human T Lymphocyte Cytoskeleton by the Cytolinker Plectin.
M. J. Brown, J. A. Hallam, Y. Liu, K. M. Yamada, and S. Shaw (2001)
J. Immunol. 167, 641-645
   Abstract »    Full Text »    PDF »
Worms reveal essential functions for intermediate filaments.
R. D. Goldman (2001)
PNAS 98, 7659-7661
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Mechanical properties and structure of carotid arteries in mice lacking desmin.
P. Lacolley, P. Challande, S. Boumaza, G. Cohuet, S. Laurent, P. Boutouyrie, J.-A. Grimaud, D. Paulin, J.-M. D. Lamaziere, and Z. Li (2001)
Cardiovasc Res 51, 178-187
   Abstract »    Full Text »    PDF »
Rigidity of Circulating Lymphocytes Is Primarily Conferred by Vimentin Intermediate Filaments.
M. J. Brown, J. A. Hallam, E. Colucci-Guyon, and S. Shaw (2001)
J. Immunol. 166, 6640-6646
   Abstract »    Full Text »    PDF »
Complete Cytolysis and Neonatal Lethality in Keratin 5 Knockout Mice Reveal Its Fundamental Role in Skin Integrity and in Epidermolysis Bullosa Simplex.
B. Peters, J. Kirfel, H. Bussow, M. Vidal, and T. M. Magin (2001)
Mol. Biol. Cell 12, 1775-1789
   Abstract »    Full Text »    PDF »
Keratin 8 Mutations in Patients with Cryptogenic Liver Disease.
N.-O. Ku, R. Gish, T. L. Wright, and M. B. Omary (2001)
N. Engl. J. Med. 344, 1580-1587
   Abstract »    Full Text »    PDF »
Insights into the Dynamic Properties of Keratin Intermediate Filaments in Living Epithelial Cells.
K. H. Yoon, M. Yoon, R. D. Moir, S. Khuon, F. W. Flitney, and R. D. Goldman (2001)
J. Cell Biol. 153, 503-516
   Abstract »    Full Text »    PDF »
Bridging cytoskeletal intersections.
E. Fuchs and I. Karakesisoglou (2001)
Genes & Dev. 15, 1-14
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