Col6a1 Null Mice as a Model to Study Skin Phenotypes in Patients with Collagen VI Related Myopathies: Expression of Classical and Novel Collagen VI Variants during Wound Healing

Lettmann, Sandra; Bloch, Wilhelm; Maaß, Tobias; Niehoff, Anja; Schulz, Jan-Niklas; Eckes, Beate; Eming, Sabine A.; Bonaldo, Paolo; Paulsson, Mats; Wagener, Raimund

doi:10.1371/journal.pone.0105686

Cited by 43 publications

(39 citation statements)

References 38 publications

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“…In contrast to the broader distribution of the α1(VI), α2(VI) and α3(VI) chains in skin, α5(VI) is found to be localized in the papillary dermis, whereas α6(VI) is found around the blood vessels . It has been reported that variants of COL6A5 are associated with atopic dermatitis (Söderhäll et al, 2007); however, work in Col6a1 −/− mice has not revealed any overt skin or wound healing defect, apart from a decrease in tensile strength (Lettmann et al, 2014). In a very recent study, we have demonstrated the involvement of ColVI in hair growth and maturation.…”

Section: Skinmentioning

confidence: 49%

“…Not surprisingly, several skin abnormalities, such as keloids or 'cigarette paper' scars, dry skin, striae rubrae and follicular keratosis, have been described in individuals with mutations in the COL6A1, COL6A2 or COL6A3 genes Lettmann et al, 2014). A recent study also describes some COL6A6 variants that are likely to be pathogenic, but functional studies are still required to confirm their role in disease (Hunter et al, 2015).…”

Section: Skinmentioning

confidence: 99%

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Collagen VI at a glance

Cescon

Gattazzo

Chen

et al. 2015

Journal of Cell Science

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283

286

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Collagen VI represents a remarkable extracellular matrix molecule, and in the past few years, studies of this molecule have revealed its involvement in a wide range of tissues and pathological conditions. In addition to its complex multi-step pathway of biosynthesis and assembly that leads to the formation of a characteristic and distinctive network of beaded microfilaments in the extracellular matrix, collagen VI exerts several key roles in different tissues. These range from unique biomechanical roles to cytoprotective functions in different cells, including myofibers, chondrocytes, neurons, fibroblasts and cardiomyocytes. Indeed, collagen VI has been shown to exert a surprisingly broad range of cytoprotective effects, which include counteracting apoptosis and oxidative damage, favoring tumor growth and progression, regulating autophagy and cell differentiation, and even contributing to the maintenance of stemness. In this Cell Science at a Glance article and the accompanying poster, we present the current knowledge of collagen VI, and in particular, discuss its relevance in stemness and in preserving the mechanical properties of tissues, as well as its links with human disorders.

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Section: Skinmentioning

confidence: 49%

Section: Skinmentioning

confidence: 99%

Collagen VI at a glance

Cescon

Gattazzo

Chen

et al. 2015

Journal of Cell Science

Self Cite

283

286

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“…15 Examination of soluble collagen VI synthesis by HFFs revealed a similar release profile to that seen for collagen I, with all peroxidases tested inducing a response that was equal to, or greater than, that seen with AA stimulation ( Figure 1B). Throughout these experiments, the peroxidase proteins had no impact on the proliferation or viability of the confluent fibroblasts (data not shown).…”

Section: Peroxidases Regulate Collagenmentioning

confidence: 60%

Peroxidase Enzymes Regulate Collagen Extracellular Matrix Biosynthesis

DeNichilo

Panagopoulos

Rayner

et al. 2015

The American Journal of Pathology

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Myeloperoxidase and eosinophil peroxidase are heme-containing enzymes often physically associated with fibrotic tissue and cancer in various organs, without any direct involvement in promoting fibroblast recruitment and extracellular matrix (ECM) biosynthesis at these sites. We report herein novel findings that show peroxidase enzymes possess a well-conserved profibrogenic capacity to stimulate the migration of fibroblastic cells and promote their ability to secrete collagenous proteins to generate a functional ECM both in vitro and in vivo. Mechanistic studies conducted using cultured fibroblasts show that these cells are capable of rapidly binding and internalizing both myeloperoxidase and eosinophil peroxidase. Peroxidase enzymes stimulate collagen biosynthesis at a post-translational level in a prolyl 4-hydroxylase-dependent manner that does not require ascorbic acid. This response was blocked by the irreversible myeloperoxidase inhibitor 4-amino-benzoic acid hydrazide, indicating peroxidase catalytic activity is essential for collagen biosynthesis. These results suggest that peroxidase enzymes, such as myeloperoxidase and eosinophil peroxidase, may play a fundamental role in regulating the recruitment of fibroblast and the biosynthesis of collagen ECM at sites of normal tissue repair and fibrosis, with enormous implications for many disease states where infiltrating inflammatory cells deposit peroxidases.

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“…IC50 denotes the concentration of sample (mg/mL) required to inhibit 50% of enzyme activity. IC50 were estimated by non-linear regression analysis employing the program GraFit (Leatherbarrow, 1998). All determinations and assays were carried out in triplicate and at least three replicates of each experiment were performed.…”

Section: Enzyme Inhibition Assaysmentioning

confidence: 99%

Skin Protective Effects of Nannochloropsis gaditana Extract on H2O2-Stressed Human Dermal Fibroblasts

Letsiou¹,

Kalliampakou

Gardikis³

et al. 2017

Front. Mar. Sci.

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Nowadays, there is huge interest in natural products obtained from marine organisms that can promote a state of health and well-being for humans. Microalgae represent a primary source of bioactive compounds that could be used as functional ingredients in cosmetic formulations. The aim of the present study is to evaluate, for the first time, the effects of Nannochloropsis gaditana extract against oxidative stress in human primary fibroblasts so as to investigate the potential applications of it in cosmetics. To gain an insight into the molecular mechanisms of N. gaditana bioactivity, we developed a new RT-qPCR platform for studying transcript accumulation for an array of selected genes (up to 100) involved in many skin-related processes including anti-aging, hydration, oxidative stress response, and DNA damage. For the oxidative stress evaluation, H 2 O 2 was used as a stressor. The study of the transcript accumulation of genes revealed that N. gaditana extract exhibits skin protection properties by mediating oxidative responses and apoptosis (including SOD1, GPX1, BID), positively regulates genes involves in skin texture and hydration (including AQP3, Col6A1, FBN1) and modulates the expression of genes involved in skin irritation, DNA damage and aging (including IL1R, PCNA, FOXO3). These findings indicate that the specific N. gaditana extract possesses significant in vitro skin protection activity against induced oxidative stress, and provide new insights into the beneficial role of microalgae bioactive compounds in cosmetic formulations protecting skin from oxidative stress.

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Col6a1 Null Mice as a Model to Study Skin Phenotypes in Patients with Collagen VI Related Myopathies: Expression of Classical and Novel Collagen VI Variants during Wound Healing

Cited by 43 publications

References 38 publications

Collagen VI at a glance

Collagen VI at a glance

Peroxidase Enzymes Regulate Collagen Extracellular Matrix Biosynthesis

Skin Protective Effects of Nannochloropsis gaditana Extract on H2O2-Stressed Human Dermal Fibroblasts

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