ArticleThe Journal of cell biology1991
A function for keratins and a common thread among different types of epidermolysis bullosa simplex diseases.
Article in The Journal of cell biology, 1991. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 66 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
66 citing papers in PubMed, 201 citations in OpenAlex.
- Extracellular matrix in skin diseases: The road to new therapies.Journal of advanced research · 2023Review
- Growth, lifetime, directional movement and myosin-dependent motility of mutant keratin granules in cultured cells.Scientific reports · 2021Article
- A Scarless Healing Tale: Comparing Homeostasis and Wound Healing of Oral Mucosa With Skin and Oesophagus.Frontiers in cell and developmental biology · 2021Review
- Keratin 14-dependent disulfides regulate epidermal homeostasis and barrier function via 14-3-3σ and YAP1.eLife · 2020Article
- Pathophysiology of pachyonychia congenita-associated palmoplantar keratoderma: new insights into skin epithelial homeostasis and avenues for treatment.The British journal of dermatology · 2020Review
- Known and novel mutations responsible for epidermolysis bullosa simplex cases in a Chinese population.Experimental and therapeutic medicine · 2019Article
- Synthetic tissues.Emerging topics in life sciences · 2019Article
- Unraveling the ECM-Immune Cell Crosstalk in Skin Diseases.Frontiers in cell and developmental biology · 2019Review
- Skin Stem Cells in Silence, Action, and Cancer.Stem cell reports · 2018Review
- Types I and II Keratin Intermediate Filaments.Cold Spring Harbor perspectives in biology · 2018Review
- Mechanical and signaling roles for keratin intermediate filaments in the assembly and morphogenesis ofDevelopment (Cambridge, England) · 2017Article
- The Molecular Revolution in Cutaneous Biology: Keratin Genes and their Associated Disease: Diversity, Opportunities, and Challenges.The Journal of investigative dermatology · 2017Review
- A novelExperimental and therapeutic medicine · 2016Article
- Discovery of keratin function and role in genetic diseases: the year that 1991 was.Molecular biology of the cell · 2016Review
- Oxidative stress and dysfunctional NRF2 underlie pachyonychia congenita phenotypes.The Journal of clinical investigation · 2016Article
- Skin Keratins.Methods in enzymology · 2016Article
- Cytokeratin 8 in Association with sdLDL and ELISA Development.North American journal of medical sciences · 2015Article
- Distinct Impact of Two Keratin Mutations Causing Epidermolysis Bullosa Simplex on Keratinocyte Adhesion and Stiffness.The Journal of investigative dermatology · 2015Article
- Skin fragility and impaired desmosomal adhesion in mice lacking all keratins.The Journal of investigative dermatology · 2014Article
- Defining keratin protein function in skin epithelia: epidermolysis bullosa simplex and its aftermath.The Journal of investigative dermatology · 2012Review
6 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Previously we demonstrated that transgenic mice expressing a mutant keratin in the basal layer of their stratified squamous epithelia exhibited a phenotype bearing resemblance to a subclass (Dowling Meara) of a heterogeneous group of human skin disorders known as epidermolysis bullosa simplex (EBS) (Vassar, R., P. A. Coulombe, L. Degenstein, K. Albers, E. Fuchs. 1991. Cell. 64:365-380.). The extent to which subtypes of EBS diseases might be genetically related is unknown, although they all exhibit skin blistering as a consequence of basal cell cytolysis. We have now examined transgenic mice expressing a range of keratin mutants which perturb keratin filament assembly to varying degrees. We have generated phenotypes which include most subtypes of EBS, demonstrating for the first time that at least in mice, these diseases can be generated by different mutations within a single gene. A strong correlation existed between the severity of the disease and the extent to which the keratin filament network was disrupted, implicating perturbations in keratin networks as an essential component of these diseases. Some keratin mutants elicited subtle perturbations, with no signs of the tonofilament clumping typical of Dowling-Meara EBS and our previous transgenic mice. Importantly, basal cell cytolysis still occurred, thereby uncoupling cytolysis from the generation of large, insoluble cytoplasmic protein aggregates. Moreover, cell rupture occurred in a narrowly defined subnuclear zone, and seemed to involve three factors: (a) filament perturbation, (b) the columnar shape of the basal cell, and (c) physical trauma. This work provides the best evidence to date for a structural function of a cytoplasmic intermediate filament network, namely to impart mechanical integrity to the cell in the context of its tissue.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.