Keith R. Porter Lecture, 1996. Of mice and men: genetic disorders of the cytoskeleton.
Open Access
- 1 February 1997
- journal article
- lecture
- Published by American Society for Cell Biology (ASCB) in Molecular Biology of the Cell
- Vol. 8 (2) , 189-203
- https://doi.org/10.1091/mbc.8.2.189
Abstract
Since the time when I was a postdoctoral fellow under the supervision of Dr. Howard Green, then at the Massachusetts Institute of Technology, I have been interested in understanding the molecular mechanisms underlying growth, differentiation, and development in the mammalian ectoderm. The ectoderm gives rise to epidermal keratinocytes and to neurons, which are the only two cell types of the body that devote most of their protein-synthesizing machinery to developing an elaborate cytoskeletal architecture composed of 10-nm intermediate filaments (IFs). Our interest is in understanding the architecture of the cytoskeleton in keratinocytes and in neurons, and in elucidating how perturbations in this architecture can lead to degenerative diseases of the skin and the nervous system. I will concentrate on the intermediate filament network of the skin and its associated genetic disorders, since this has been a long-standing interest of my laboratory at the University of Chicago.Keywords
This publication has 97 references indexed in Scilit:
- Absence of integrin α6 leads to epidermolysis bullosa and neonatal death in miceNature Genetics, 1996
- Chronic hepatitis, hepatocyte fragility, and increased soluble phosphoglycokeratins in transgenic mice expressing a keratin 18 conserved arginine mutant.The Journal of cell biology, 1995
- Integrin β4 mutations associated with junctional epidermolysis bullosa with pyloric atresiaNature Genetics, 1995
- INTERMEDIATE FILAMENTS: Structure, Dynamics, Function and DiseaseAnnual Review of Biochemistry, 1994
- Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: A mouse model of amyotrophic lateral sclerosisCell, 1993
- Keratin Intermediate Filament Structure: Crosslinking Studies Yield Quantitative Information on Molecular Dimensions and Mechanism of AssemblyJournal of Molecular Biology, 1993
- A family of type I keratin genes and the homeobox-2 gene complex are closely linked to the rex locus on mouse chromosome 11Genomics, 1989
- The cDNA sequence of a type II cytoskeletal keratin reveals constant and variable structural domains among keratinsCell, 1983
- Two distinct classes of keratin genes and their evolutionary significanceCell, 1981
- Structure of α-keratin: Structural implication of the amino acid sequences of the type I and type II chain segmentsJournal of Molecular Biology, 1977