ReviewInternational journal of molecular sciences2021
Keratins as an Inflammation Trigger Point in Epidermolysis Bullosa Simplex.
Review in International journal of molecular sciences, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 43 citations in OpenAlex.
- Keratin-associated epidermolysis bullosa simplex: phenotypes and challenges in clinical trials - a narrative review and systematic update.Orphanet journal of rare diseases · 2025Pooled it
- Impact of Microgravity on Cytoskeletal Dynamics, Protein Transport, and Signaling Networks: Potential Therapeutic Opportunities for Skin Health.International journal of molecular sciences · 2026Review
- MSC-Derived Secretome and Exosomes in Dermatology: Mechanisms, Therapeutic Opportunities, and Scientific Challenges-A Narrative Review.International journal of dermatology · 2026Review
- An isogenic hiPSC-derived keratinocyte model reveals CXCL10/CXCL11 inflammatory dysregulation in epidermolysis bullosa simplex.Frontiers in cell and developmental biology · 2026Article
- Living donor kidney transplantation in a patient with inherited skin fragility disorder in a resource-limited setting: a case report.Journal of surgical case reports · 2025Article
- Cutaneous Squamous Cell Carcinoma in Epidermolysis Bullosa: A Review of Pathogenesis, Diagnosis and Management.Cancers · 2025Review
- Pemphigus Vulgaris Autoantibodies Induce an Endoplasmic Reticulum Stress Response.The Journal of investigative dermatology · 2025Article
- Adipose tissue-associated Krt23+fibroblasts contribute to immune microenvironment disorders in mouse adipose tissue during the development of obesity.European journal of medical research · 2025Article
- Hypoxia-induced RHCG as a key regulator in psoriasis and its modulation by secukinumab.APL bioengineering · 2025Article
- Virucidal and Bactericidal Properties of Biocompatible Copper Textiles.Global challenges (Hoboken, NJ) · 2025Article
- Intermediate filaments and their associated molecules.Journal of biomedical research · 2025Article
- Expression of an epidermal growth factor-transdermal peptide fusion protein inFrontiers in plant science · 2025Article
- Identification and culture of functional salivary gland ductal epithelial cells.Histochemistry and cell biology · 2024Article
- Spatiotemporal characterization of extracellular matrix maturation in human artificial stromal-epithelial tissue substitutes.BMC biology · 2024Article
- A statistical mechanics investigation of unfolded protein response across organisms.Scientific reports · 2024Article
- Pathological Mechanisms Involved in Epidermolysis Bullosa Simplex: Current Knowledge and Therapeutic Perspectives.International journal of molecular sciences · 2024Review
- Small fiber neuropathy in epidermolysis bullosa simplex.JAAD case reports · 2024Article
- Architecture and dynamics of a desmosome-endoplasmic reticulum complex.Nature cell biology · 2023Article
- Stairways to Advanced Therapies for Epidermolysis Bullosa.Cold Spring Harbor perspectives in biology · 2023Review
- A Kaleidoscope of Keratin Gene Expression and the Mosaic of Its Regulatory Mechanisms.International journal of molecular sciences · 2023Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 2 institutions in 1 country.
Funding
Abstract
Epidermolysis bullosa simplex (EBS) is a group of inherited keratinopathies that, in most cases, arise due to mutations in keratins and lead to intraepidermal ruptures. The cellular pathology of most EBS subtypes is associated with the fragility of the intermediate filament network, cytolysis of the basal layer of the epidermis, or attenuation of hemidesmosomal/desmosomal components. Mutations in keratins 5/14 or in other genes that encode associated proteins induce structural disarrangements of different strengths depending on their locations in the genes. Keratin aggregates display impaired dynamics of assembly and diminished solubility and appear to be the trigger for endoplasmic reticulum (ER) stress upon being phosphorylated by MAPKs. Global changes in cellular signaling mainly occur in cases of severe dominant EBS mutations. The spectrum of changes initiated by phosphorylation includes the inhibition of proteasome degradation, TNF-α signaling activation, deregulated proliferation, abnormal cell migration, and impaired adherence of keratinocytes. ER stress also leads to the release of proinflammatory danger-associated molecular pattern (DAMP) molecules, which enhance avalanche-like inflammation. Many instances of positive feedback in the course of cellular stress and the development of sterile inflammation led to systemic chronic inflammation in EBS. This highlights the role of keratin in the maintenance of epidermal and immune homeostasis.
Indexed as
Identifiers
What OpenQuestion holds
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.