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Previous Volume 351:2087-2100 November 11, 2004 Number 20 Next

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The cytoskeleton consists of three abundant families of fibrillary proteins: microfilaments, microtubules, and intermediate filaments.^1,2 Intermediate filament proteins derive their name from their diameter, which is intermediate between the diameters of microfilaments and microtubules.^1,2 They differ from actin microfilaments and tubulin microtubules in their large number, their distribution in the cytoplasm and nucleus, their diverse primary structure (Table 1), their nonpolar architecture, their relative insolubility, and their nucleotide-independent dynamics.^1,2,3 The human genome contains at least 65 functional genes encoding intermediate filament proteins, placing them among the 100 largest gene families in humans.⁴ More than 30 diseases are related . . . [Full Text of this Article]

General Features of Intermediate Filaments

Structure

Regulation

Functions

Shared and Tissue-Specific Functions

Disease-Related Aspects

Cytoplasmic Intermediate Filament–Related Diseases

Diseases of the Skin and Epithelium

Diseases of the Hair

Myopathies and Lens Disorders

Neurodegenerative Disorders

Interacting-Protein–Related Diseases

Pathogenesis of Cytoplasmic Intermediate Filament–Related Diseases

Organization and Redundancy

Cell Fragility

Disruption of Organelle and Protein Targeting

Genetics, Epigenetics, and Environment

Laminopathies

Diseases

Pathogenesis

Therapeutic Approaches

Future Perspectives

Source Information

From the Department of Medicine, Palo Alto Veterans Affairs Medical Center and Stanford University, Palo Alto, Calif. (M.B.O.); the Departments of Biological Chemistry and Dermatology, Johns Hopkins University School of Medicine, Baltimore (P.A.C.); and the Human Genetics Unit, University of Dundee, Ninewells Hospital and Medical School, Dundee, United Kingdom (W.H.I.M.).

Address reprint requests to Dr. Omary at the Palo Alto VA Medical Center, 3801 Miranda Ave., Mail Code 154J, Palo Alto, CA 94304.

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