Abstract:
:The protease inhibitor cystatin M/E (CST6) regulates a biochemical pathway involved in stratum corneum homeostasis, and its deficiency in mice causes ichthyosis and neonatal lethality. Cystatin M/E deficiency has not been described in humans so far, and we did not detect disease-causing mutations in the CST6 gene in a large number of patients with autosomal recessive congenital ichthyosis, who were negative for mutations in known ichthyosis-associated genes. To investigate the phenotype of CST6 deficiency in human epidermis, we used lentiviral delivery of short hairpin RNAs that target CST6 in a 3D reconstructed skin model. Surprisingly, CST6 deficiency did not cause an ichthyosis-like phenotype, but prevented the development of a multilayered epidermis. From this study, we conclude that CST6 deficiency may be incompatible with normal human foetal development.
journal_name
Exp Dermatoljournal_title
Experimental dermatologyauthors
Jansen PA,van den Bogaard EH,Kersten FF,Oostendorp C,van Vlijmen-Willems IM,Oji V,Traupe H,Hennies HC,Schalkwijk J,Zeeuwen PLdoi
10.1111/exd.12022subject
Has Abstractpub_date
2012-11-01 00:00:00pages
889-91issue
11eissn
0906-6705issn
1600-0625journal_volume
21pub_type
信件abstract::Primary cutaneous large B-cell lymphomas, leg type (PCLBCL/LT) are primary cutaneous B-cell lymphoma (PCBCL) with an intermediate prognosis. Therefore, antracycline-based polychemotherapy combined with rituximab has been recommended as first-line treatment. Yet, despite this regimen, the 5-year survival rate remains 5...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.12688
更新日期:2015-06-01 00:00:00
abstract::This study demonstrates an innovative application of atomic force microscopy (AFM). The combination of high-resolution AFM technology and tape stripping is presented as a tool for the structure analysis of human stratum corneum (SC) at a nanometer scale. Topographic images with a vertical resolution of about 10 nm of ...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.0906-6705.2006.00424.x
更新日期:2006-05-01 00:00:00
abstract::The contribution of immune checkpoint receptors in the immunopathogenesis of various autoimmune diseases has been addressed in previous reports. In this study, the expression profile of T-cell immunoglobulin and mucin-domain containing-3 (Tim-3) and programmed cell death-1 (PD-1) checkpoint molecules was investigated...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.13952
更新日期:2019-06-01 00:00:00
abstract::Rosacea is one of the most common dermatoses of adults. Although the detailed pathophysiology remains unknown, it is thought that rosacea is caused by a consistently aberrant, innate immune response, and that LL-37 plays an important role. However, involvement of the inflammatory cytokine IL-33 has not yet been studie...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.13702
更新日期:2018-09-01 00:00:00
abstract::Patients belonging to xeroderma pigmentosum (XP) complementation group C comprise one-third of all XP patients. Only four major reports compiled larger groups of XP-C patients from southern Europe (12 pts), North America (16 pts) and Africa (14 and 56 pts) as well as their genetic background (46 XPC mutations). We ide...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.12052
更新日期:2013-01-01 00:00:00
abstract:BACKGROUND:Recently, germline variants of the melanocortin-1 receptor (MC1R) have been shown to be associated with an increased risk for BRAF mutant but not BRAF wild-type cutaneous melanoma. Similar to melanoma, BRAF mutations are also commonly found in papillary thyroid carcinomas. Furthermore, patients with melanoma...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2008.00827.x
更新日期:2009-06-01 00:00:00
abstract::We aimed to evaluate the effect of small interfering RNA (siRNA) targeting CTGF on extracellular matrices (ECMs) metabolism in normal and SSc fibroblasts. Normal and SSc fibroblasts were transfected with CTGF-specific siRNAs to silence CTGF synthesis. After silencing CTGF, production of type I collagen and matrix meta...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2009.00999.x
更新日期:2010-08-01 00:00:00
abstract::Pemphigus foliaceus (PF) is a severe autoimmune bullous disorder, characterized by autoantibodies (autoAb) against desmoglein 1 (Dsg1). As T cells may be critical in the pathology of PF, the aim of the present study was to identify and characterize autoaggressive T-helper cells reactive to Dsg1 in PF patients and heal...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.0906-6705.2005.00329.x
更新日期:2005-08-01 00:00:00
abstract::Host defense peptides/proteins (HDPs), also known as antimicrobial peptides/proteins (AMPs), are key molecules in the cutaneous innate immune system. AMPs/HDPs historically exhibit broad-spectrum killing activity against bacteria, enveloped viruses, fungi and several parasites. Recently, AMPs/HDPs were shown to have i...
journal_title:Experimental dermatology
pub_type: 杂志文章,评审
doi:10.1111/exd.13314
更新日期:2017-11-01 00:00:00
abstract::Sangre de drago (SD) is a viscous bright red resin collected from Croton lechleri trees that grow in the South American jungle. This sap is used extensively in the native pharmacopoeia to treat skin disorders. Its effectiveness as an inhibitor of neurogenic inflammation has been recently demonstrated. To understand th...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2010.01090.x
更新日期:2010-09-01 00:00:00
abstract::T cells play a major role in inflammatory skin disorders such as psoriasis vulgaris and atopic dermatitis. They are both active on the level of cell-to-cell interaction and by the secretion of pro-inflammatory mediators. CD26 is a lymphocyte membrane-associated dipeptidyl peptidase IV (DPP IV), which is able to inacti...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1034/j.1600-0625.2001.100604.x
更新日期:2001-12-01 00:00:00
abstract::Acquisition of potent human dermal papilla (DP) cells that can induce hair follicle neogenesis is an overarching concern, and various approaches have been tried. In an attempt to solve the problem, we previously introduced the three-dimensional (3D) culture of human DP cells and observed de novo formation of hair foll...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.13234
更新日期:2017-02-01 00:00:00
abstract::Psoriasis is now considered to be a chronic, immune-mediated and inflammatory skin disease. As the precise cause of psoriasis remains unknown, its treatment is challenging for dermatologists. Keratin 17 (K17), an intermediate filament protein, is highly expressed in psoriatic lesions, while not normally expressed in h...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2010.01235.x
更新日期:2011-07-01 00:00:00
abstract::Antibodies, specific to murine DEC205, can be used to target antigens to dendritic cells. The immunodominant domain of human type XVII collagen, hNC16A, was fused to this antibody (DEC-hNC16A) and was administered as expression plasmid by gene gun transfection with the aim of inducing tolerance to human type XVII coll...
journal_title:Experimental dermatology
pub_type: 信件
doi:10.1111/j.1600-0625.2012.01474.x
更新日期:2012-05-01 00:00:00
abstract::Collagens are the most abundant proteins in the extracellular matrix. They provide a framework to build organs and tissues and give structural support to make them resistant to mechanical load and forces. Several intra- and extracellular modifications are needed to make functional collagen molecules, intracellular pos...
journal_title:Experimental dermatology
pub_type: 杂志文章,评审
doi:10.1111/exd.14197
更新日期:2021-01-01 00:00:00
abstract::The pathogenesis of lichen sclerosus remains unknown. However, it has been frequently associated clinically with autoimmunity. The MHC haplotype A1, B8, DR3 is associated with many autoimmune conditions and has also been associated with the uncommon allele of the tumour necrosis factor (TNF-alpha) promoter polymorphis...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.1996.tb00121.x
更新日期:1996-08-01 00:00:00
abstract::Bullous pemphigoid (BP) and pemphigoid gestationis (PG) are acquired autoimmune subepidermal blistering diseases characterized by autoantibodies against the hemidesmosomal proteins BP180/type XVII collagen and BP230. In the vast majority of BP and PG patients, these autoantibodies bind to epitopes clustered within the...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2007.00592.x
更新日期:2007-09-01 00:00:00
abstract::Hair follicles cyclically regenerate throughout adult mammalian life, owing to a resident population of epithelial hair follicle stem cells. Stem cell (SC) activity drives bouts of follicle growth, which are periodically interrupted by follicle regression and rest. These phases and the transitions between them are tig...
journal_title:Experimental dermatology
pub_type: 杂志文章,评审
doi:10.1111/exd.14204
更新日期:2020-10-02 00:00:00
abstract::Rosacea is a skin inflammatory condition accompanied by cutaneous signs such as oedema, flushing, erythema, telangiectasia and pustules. Generally, rosacea is triggered by ultraviolet B (UVB) exposure. When exposed to UVB, skin epidermis thickens and produces elevated levels of pro-inflammatory cytokines, especially k...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.14115
更新日期:2020-05-20 00:00:00
abstract::Proteases and their receptors have poorly understood roles in skin fibrosis and systemic scleroderma (SSc). We examined the role of protease-activated receptors (PAR(1) and PAR(2) ) in the pathophysiology of human SSc and skin fibrosis. Immunohistochemistry showed that PAR(1) immunoreactivity was positive in fibroblas...
journal_title:Experimental dermatology
pub_type: 信件
doi:10.1111/j.1600-0625.2010.01184.x
更新日期:2011-01-01 00:00:00
abstract::A number of single gene mutations in laboratory mice produce hair follicle defects resulting in deformed hair shafts. The radiation-induced (SB/LeJ-Foxq1(sa)) satin mutant mice have a satin-like sheen to their hair and dilute colouration. This sheen is due to failure of the hair shafts to develop normal medullas, whil...
journal_title:Experimental dermatology
pub_type: 信件
doi:10.1111/exd.12106
更新日期:2013-03-01 00:00:00
abstract::Type-1 cytokine-producing T cells are important in the pathogenesis of psoriasis vulgaris, for which efficient therapy is provided by means of narrow-band ultraviolet-B (NB-UVB). The expression of the type-1 cytokine interferon-gamma (IFN-gamma) is regulated by interleukin-12 (IL-12), IL-15, IL-18 and IL-23; however, ...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.0906-6705.2004.00246.x
更新日期:2004-12-01 00:00:00
abstract::Inflammation is a crucial step in the development of allergic contact dermatitis. The primary contact with chemical allergens, called sensitization, and the secondary contact, called elicitation, result in an inflammatory response in the skin. The ability of contact allergens to induce allergic contact dermatitis corr...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2010.01136.x
更新日期:2010-11-01 00:00:00
abstract::The adoptive transfer of engineered T cells represents an important approach in immunotherapy of melanoma. However, relapse of the tumor can occur due to immune-escape mechanisms developed by the tumor cells, for example antigen loss, downregulation of the major histocompatibility complex presentation machinery and de...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.13095
更新日期:2016-11-01 00:00:00
abstract::White adipose tissue is known to be involved in numerous physiological processes such as insulin-mediated functions, lipid and glucose metabolism, vascular changes and coagulation. These processes are mainly mediated by adipokines that are secreted either from adipocytes or cells of the stromal-vascular fraction of ad...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/j.1600-0625.2011.01402.x
更新日期:2012-01-01 00:00:00
abstract::More than 40 null mutations in the filaggrin (FLG) gene are described. It is therefore possible to find two different null mutations in one individual (compound heterozygosity). It has been generally perceived that homozygous and compound heterozygous individuals were genotypically comparable; however, this has not be...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.12199
更新日期:2013-09-01 00:00:00
abstract::Chronic pruritus (CP) is defined by itching lasting 6 weeks or more and is known to be both a highly prevalent and burdensome symptom of many pruritogenic diseases. Its status as a symptom has many implications mainly that the symptom should evolve and subside with the disease. However, according to the clinical exper...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.13953
更新日期:2019-12-01 00:00:00
abstract::Claudins (Cldn) are the major components of tight junctions (TJs) sealing the paracellular cleft in tissue barriers of various organs. Zebrafish Cldnb, the homolog of mammalian Cldn4, is expressed at epithelial cell-cell contacts and is important for regulating epidermal permeability. The bacterial toxin Clostridium p...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.12728
更新日期:2015-08-01 00:00:00
abstract::Canine atopic dermatitis (AD), a chronic inflammatory skin disease, shares characteristics with its human counterpart. To get insight into the role of enzymes involved in production of prostaglandin E(2) (PGE2) and leukotriene B(4) (LTB(4)), potent inflammatory mediators originating from membrane-derived arachidonic a...
journal_title:Experimental dermatology
pub_type: 信件
doi:10.1111/j.1600-0625.2009.01037.x
更新日期:2010-08-01 00:00:00
abstract::Flavonoids belong to a large group of polyphenolic compounds that are widely present in plants. Certain flavonoids, including naringenin, have cytoprotective properties. Although the antioxidant effect has long been thought to be a crucial factor accounting for the cellular effects of flavonoids, mitochondrial channel...
journal_title:Experimental dermatology
pub_type: 杂志文章
doi:10.1111/exd.13903
更新日期:2019-05-01 00:00:00