Meanwhile, European researchers identified KLHL24 mutations in families from Finland, Germany, Israel, Italy, Switzerland, and Qatar and confirmed that these mutations affect the equilibrium between intermediate
keratin filaments and keratin breakdown that is necessary for skin integrity (Am J Hum Genet.
They contain
keratin filaments, filaggrin (protein surrounding
keratin filaments) and the natural moisturizing factor (NMF), elements that confer flexibility and mechanical resilience to the SC.
In BDD, cows develop lesions with long
keratin filaments in skin just above the hooves.
(2) An important aspect of stratified squamous epithelia is that the cells undergo a terminal differentiation program that results in the formation of a mechanically resistant and toughened surface composed of cornified cells that are filled with
keratin filaments and lack nuclei and cytoplasmic organelles.
Filaggrin proteins cause
keratin filaments in the epidermis to aggregate in ways that prevent epidermal water loss and impede entry of unwanted environmental chemicals, haptens, and proteins.
Keratosis usually features a total replacement of superficial epithelial cells by
keratin filaments and by dissolution of the nuclei.
Also known as filament aggregating protein, filaggrin consolidates
keratin filaments into dense bundles in the granular layer of the epidermis during the terminal phase of epithelial differentiation, forming a tight matrix that also maintains moisture within the skin, he explained.
The mutation might have impaired the liver's ability to reorganize
keratin filaments in response to physiologic stress.
GREEN: What are the differences in mechanical properties between
keratin filaments and type III filaments?
The researchers thought this protein linked the intricate network of
keratin filaments inside each skin cell to certain molecules in the cell membrane.
Onset of re-epithelialization after skin injury correlates with a reorganization of
keratin filaments in wound edge keratinocytes: defining a potential role for keratin 16.
We have calculated what this means in terms of the extent of cross-linking of
keratin filaments. Assuming that
keratin filaments are 15 nm wide, and assuming a model in which the filaments completely line the intracellular surface of the cell envelope, we calculate about one crosslink per 100 nm of filament length.
