Quasi-normal cornified cell envelopes in loricrin knockout mice imply the existence of a loricrin backup system.

@article{Jarnik2002QuasinormalCC,
  title={Quasi-normal cornified cell envelopes in loricrin knockout mice imply the existence of a loricrin backup system.},
  author={M. Jarnik and P. D. de Viragh and E. Sch{\"a}rer and D. Bundman and M. Simon and D. Roop and A. Steven},
  journal={The Journal of investigative dermatology},
  year={2002},
  volume={118 1},
  pages={
          102-9
        }
}
The cornified cell envelope, a lipoprotein layer that assembles at the surface of terminally differentiated keratinocytes, is a resilient structure on account of covalent crosslinking of its constituent proteins, principally loricrin, which accounts for up to 60%-80% of total protein. Despite the importance of the cell envelope as a protective barrier, knocking out the loricrin gene in mice results in only mild syndromes. We have investigated the epidermis and forestomach epithelium of these… Expand
Proteomic Analysis of Loricrin Knockout Mouse Epidermis.
TLDR
The results suggest that loss of loricrin alters their incorporation into envelopes as a consequence of protein-protein interactions during cell maturation, and helps rationalize the potential formation of functional envelopes, despite loss of a single component, due to the availability of many alternative transglutaminase substrates. Expand
Loricrin: Past, Present, and Future
TLDR
Through an extensive review of aggregate evidence, the functional significance of the thiol-rich protein loricrin from a biochemical, genetic, pathological, metabolic, metabolic or immunological aspect is discussed with some theoretical and speculative perspectives. Expand
Functional analysis of the cornified envelope precursors, periplakin and envoplakin
TLDR
It is found that the N terminus of envoplakin does not efficiently localise to desmosomes or microvilli, suggesting a dependence on periplakin for membrane localisation, and the consequence of deleting the kazrin gene in mice is interesting. Expand
Structural and functional consequences of loricrin mutations in human loricrin keratoderma (Vohwinkel syndrome with ichthyosis).
TLDR
The results show that the barrier abnormality in loricrin keratoderma is linked to a defective CE scaffold, resulting in increased extracellular permeability, as shown previously for another "scaffold disorder", lamellar ichthyosis. Expand
Periplakin Gene Targeting Reveals a Constituent of the Cornified Cell Envelope Dispensable for Normal Mouse Development
TLDR
The primary role of periplakin may not relate to the physiology of the cornified cell envelope in epidermal keratinocytes but may reside in the challenges, which normal laboratory mice do not encounter. Expand
Lce1 Family Members Are Nrf2-Target Genes that Are Induced to Compensate for the Loss of Loricrin.
TLDR
Evidence is presented suggesting that the late cornified envelope 1 proteins are also compensatory components as determined by their localization within the loricrin knockout cell envelope via immunoelectron microscopy, and it is demonstrated that late cornification envelope 1 genes are upregulated at the transcriptional level in loricin knockout mouse skin. Expand
Human stratum corneum proteomics reveals cross‐linking of a broad spectrum of proteins in cornified envelopes
TLDR
The results suggest a model where, as previously observed in hair shaft and nail plate, a diversity of cellular proteins are incorporated and help rationalize the minimal effect on epidermis of ablating genes for specific single envelope structural components. Expand
Up-regulation of Loricrin Expression by Cell Adhesion Molecule Nectin-1 through Rap1-ERK Signaling in Keratinocytes*
TLDR
Results indicate that nectin-1 plays a key role in the expression of loricrin in the epidermis, which is an immunoglobulin-like cell-cell adhesion molecule, which plays essential roles in the initial step of formation of adherens junctions and tight junctions. Expand
Caveolin expression and localization in human keratinocytes suggest a role in lamellar granule biogenesis.
TLDR
The differentiation-dependent expression of caveolin-1, the colocalization of caveolins with putative lamellar-granule-associated antigens, their enrichment in isolated lamellars granules, and their presence in lamellAR-granules-like structures on immunoelectron microscopy suggest that caveolin may play a role in lameLLar granule assembly, trafficking, and/or function. Expand
TMEM45A Is Dispensable for Epidermal Morphogenesis, Keratinization and Barrier Formation
TLDR
The TMEM45A gene expression is dispensable for epidermal morphogenesis, keratinization and barrier formation, and if this protein plays a role in the epidermis, its experimental depletion can possibly be compensated by other proteins in the two experimental models analyzed. Expand
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Protein composition of cornified cell envelopes of epidermal keratinocytes.
TLDR
The amino acid compositions of isolated CEs are determined and then modelled in terms of linear combinations of the candidate proteins, showing that stratum corneum CEs have a loricrin content of 65-70% (w/w) in human, and 80-85% in mouse. Expand
Identification of a major keratinocyte cell envelope protein, loricrin
TLDR
CDNA clones encoding a major differentiation product of mouse epidermal cells are isolated and characterized, which has an amino acid composition similar to that of purified cell envelopes and is named loricrin on the basis of its presumed function. Expand
Biochemical, Structural, and Transglutaminase Substrate Properties Of Human Loricrin, the Major Epidermal Cornified Cell Envelope Protein (*)
TLDR
The data support a hypothesis for the essential and complementary roles of both TGase 1 and TGase 3 in cross-linking of loricrin in vivo, and may explain the phenotype of lamellar ichthyosis, a disease caused by mutations in theTGase 1 gene. Expand
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TLDR
In conclusion, loricrin is initially accumulated in L-granules, to be released at a specifically programmed stage of keratinocyte maturation, and incorporated into the covalently cross-linked lining of the cell envelope, and account for the sulfur-rich keratohyalin granules described earlier. Expand
Structural organization of cornified cell envelopes and alterations in inherited skin disorders
TLDR
It is revealed that abnormal cornified cell envelope synthesis is significantly involved in the pathophysiology of certain inherited keratodermas and reflects perturbations in the complex yet highly orderly process of cornification cell envelope formation in normal skin biology. Expand
Differential expression and cell envelope incorporation of small proline-rich protein 1 in different cornified epithelia.
TLDR
Study of the CEs of two different keratinizing epithelia of mouse, with particular focus on their contents of loricrin and the small proline-rich proteins (SPRs), predicts the content ofloricrin in forestomach CEs to be very high, as in the epidermis, and accompanied by approximately 18% content of total SPRs. Expand
Mutant loricrin is not crosslinked into the cornified cell envelope but is translocated into the nucleus in loricrin keratoderma.
TLDR
Mutant loricrin, as a dominant negative disrupter, is not likely to affect cornified cell envelope crosslinking directly, but seems to interfere with nuclear/nucleolar functions of differentiating keratinocytes. Expand
Lessons from Loricrin-Deficient Mice: Compensatory Mechanisms Maintaining Skin Barrier Function in the Absence of a Major Cornified Envelope Protein
TLDR
At least one of the compensatory mechanisms preventing a more severe skin phenotype in newborn Lor−/− mice is an increase in the expression of other CE components, such as SPRRP2D and SPR RP2H, members of the family of “small proline rich proteins”, and repetin, a member of the “fused gene” subgroup of the S100 gene family. Expand
The Proteins Elafin, Filaggrin, Keratin Intermediate Filaments, Loricrin, and Small Proline-rich Proteins 1 and 2 Are Isodipeptide Cross-linked Components of the Human Epidermal Cornified Cell Envelope (*)
TLDR
The data establish for the first time that these several proteins are indeed cross-linked protein components of the CE structure, and support a model for the intermediate to final stages of CE assembly: the proteins elafin, SPR1 and SPR2, and loricrin begin to be deposited on a preformed scaffold; later,Elafin deposition decreases as loricin and SPR accumulation continues to effect final assembly. Expand
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TLDR
The results provide further evidence that the program of squamous differentiation in internal epithelia of the upper alimentary tract in rodents and higher mammals differ remarkably. Expand
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