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Titolo:
THE CYTOSKELETON AND DISEASE - GENETIC-DISORDERS OF INTERMEDIATE FILAMENTS
Autore:
FUCHS E;
Indirizzi:
UNIV CHICAGO,HOWARD HUGHES MED INST,5841 S MARYLAND AVE CHICAGO IL 60637 UNIV CHICAGO,DEPT MOL GENET & CELL BIOL CHICAGO IL 60637
Titolo Testata:
Annual review of genetics
, volume: 30, anno: 1996,
pagine: 197 - 231
SICI:
0066-4197(1996)30:<197:TCAD-G>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
EPIDERMOLYSIS-BULLOSA SIMPLEX; AMYOTROPHIC-LATERAL-SCLEROSIS; AMINO-ACID-SEQUENCE; WHITE SPONGE NEVUS; STRATIFIED SQUAMOUS EPITHELIA; EPIDERMAL KERATIN FILAMENTS; COILED-COIL MOLECULES; MOTOR-NEURON DISEASE; MUTATIONAL HOT-SPOT; TRANSGENIC MICE;
Keywords:
INTERMEDIATE FILAMENTS; CYTOSKELETON; PROTEIN STRUCTURE; GENETIC DISEASE; MULTIGENE FAMILY; PROTEIN FUNCTION;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
184
Recensione:
Indirizzi per estratti:
Citazione:
E. Fuchs, "THE CYTOSKELETON AND DISEASE - GENETIC-DISORDERS OF INTERMEDIATE FILAMENTS", Annual review of genetics, 30, 1996, pp. 197-231

Abstract

Specialized cytoskeletons play many fascinating roles, including mechanical integrity and wound-healing in epidermal cells, cell polarity in simple epithelia, contraction in muscle cells, hearing and balance in the inner ear cells, axonal transport in neurons, and neuromuscular junction formation between muscle cells and motor neurons. These varied functions are dependent upon cytoplasmic networks of actin microfilaments (6 nm), intermediate filaments (10 nm) and microtubules (23 nm),and their many associated proteins. In this chapter, I review what isknown about the cytoskeletons of intermediate filaments and their associated proteins. I focus largely on epidermal cells, which devote most of their protein-synthesizing machinery to producing an extensive intermediate filament network composed of keratin. Recent studies have shown that many of the devastating human disorders that arise from degeneration of this cell type have as their underlying basis either defects in the genes encoding keratins or abnormalities in keratin IF networks. I discuss what we know about the functions of IFs, and how the link to genetic disease hits enhanced this understanding.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/12/20 alle ore 08:03:13