Elastic Fibers Research Articles

The Role of Mast Cells in the Remodeling Effects of Molecular Hydrogen on the Lung Local Tissue Microenvironment under Simulated Pulmonary Hypertension

Molecular hydrogen (H2) has antioxidant, anti-inflammatory, and anti-fibrotic effects. In a rat model simulating pulmonary fibrotic changes induced by monocrotaline-induced pulmonary hypertension (MPH), we had previously explored the impact of inhaled H2 on lung inflammation and blood pressure. In this study, we further focused the biological effects of H2 on mast cells (MCs) and the parameters of the fibrotic phenotype of the local tissue microenvironment. MPH resulted in a significantly increased number of MCs in both the pneumatic and respiratory parts of the lungs, an increased number of tryptase-positive MCs with increased expression of TGF-β, activated interaction with immunocompetent cells (macrophages and plasma cells) and fibroblasts, and increased MC colocalization with a fibrous component of the extracellular matrix of connective tissue. The alteration in the properties of the MC population occurred together with intensified collagen fibrillogenesis and an increase in the integral volume of collagen and elastic fibers of the extracellular matrix of the pulmonary connective tissue. The exposure of H2 together with monocrotaline (MCT), despite individual differences between animals, tended to decrease the intrapulmonary MC population and the severity of the fibrotic phenotype of the local tissue microenvironment compared to changes in animals exposed to the MCT effect alone. In addition, the activity of collagen fibrillogenesis associated with MCs and the expression of TGF-β and tryptase in MCs decreased, accompanied by a reduction in the absolute and relative content of reticular and elastic fibers in the lung stroma. Thus, with MCT exposure, inhaled H2 has antifibrotic effects involving MCs in the lungs of rats. This reveals the unknown development mechanisms of the biological effects of H2 on the remodeling features of the extracellular matrix under inflammatory background conditions of the tissue microenvironment.

Light and scanning electron microscope characterization of mandibular symphysis tissue as a functional adaptation in the mandible development of human fetuses.

When developing, the mandible presents great plasticity and contains condensed mesenchymal cells that develops into Meckel's cartilage, of which the anterior part forms the mandibular symphysis. Mandible human development studies focus on investigating whether the beginning of mandibular fusion in fetal period is related to symphysis ossification and the tensions imposed on it, considering that tongue movements, mouth opening, and closing can be seen in fetuses. This research analyses tissue modifications during human mandibular symphysis growth using light and scanning electron microscopy to relate them to its functional structure. The study sample consisted of 12 human fetuses distributed into two groups: Group I (GI) of 10-14 weeks old and Group II (GII) of 20-24 weeks old. Fragments of mandibular symphysis were removed en bloc together with the surrounding tissues to preserve the relation with adjacent structures. Decalcified specimens were prepared in semi-serial coronal sections 5-μm-thick and stained with hematoxylin and eosin, Masson՚s trichrome, Verhoeff, and Sirius red for histological analysis with light microscopy. Collagen fibers Type I or III and elastic fibers were quantified by volume fraction (Vv). Coronal sections of the GI and GII symphyseal region were submitted to scanning electron microscopy. Comparison between groups used independent t-test. Our study presents the different endochondral ossification stages in the anterior part of Meckel's cartilage in GI. Both groups showed abundantly vascularized mesenchymal tissue with intense cellular activity forming the mandibular symphysis, such as a source of new osteoblasts adjacent to the newly deposited bone matrix. Scanning electron microscopy analysis revealed an invasion of the bony trabecula in the transverse direction from the hemimandible, rectilinear in GI and sinuous in GII due to interdigitating bone process, promoting its ossification. In collagen Vv analysis was verified a prevalence of type I in GII and type III in GI, indicating a proportional relation between maturation and tissue arrangement. Functionally, the collagen and elastic fibers in the mandibular symphysis were arranged in a pantographic network, and the fibrillar interconnectivity clearly contributes to resilience capacity and efficiency of the force transfer. This study inferred the functional significance of the knowledge about the tissue composition of mandibular symphysis, and the importance of this tissue for surrounding structures. The mesenchymal tissue of mandibular symphysis participates in bone growth process, revealing an adaptation mechanism of mandibular symphysis in the fetal period investigated.

EXPERIMENTAL APPROACH FOR DEVELOPMENT OF SUSTAINABLE HYBRID GRADED FIBER REINFORCED CONCRETE BY CONSUMING LATHE WASTE STEEL FIBERS WITH GLASS FIBERS FOR ENHANCED MECHANICAL PROPERTIES

Local workshops generate large quantities of industrial lathe waste steel fibers, which the steel manufacturing industries find difficult to recycle because of their sharp edges. The utilization of lathe waste steel fibers as fiber reinforcement is sustainable in concrete because these fibers have the same properties as steel fibers. Furthermore, using combinations of ductile and elastic fibers improves strain capacity and resistance to pre- and post-cracking. This research employs hybrid fiber reinforcement technology, utilizing industrial lathe waste steel fibers and glass fibers in varying proportions, to bridge the micro and macro cracks in concrete specimens. This research was done to examine the physical (workability) and mechanical properties (compressive and flexural strength) of hybrid fiber-reinforced concrete. In this research different mixtures of hybrid fiber-reinforced concrete were cast and designated as M0, M1, M2, M3, M4, M5, and M6. The mixtures included lathe waste steel fibers at 0%, 0.50%, 1%, 1.5%, 2%, 2.5%, and 3%, and glass fibers at 0%, 0.15%, 0.25%, 0.45%, 0.60%, 0.75%, and 0.90%, respectively. The ASTM-standardised protocols were followed for all laboratory testing. The physical property results showed a decrease in the workability of concrete mixes as the percentage of lathe waste steel and glass fiber increased. This suggests that a higher percentage of lathe waste steel and glass fibers leads to a lower slump value. Consequently, the mechanical property results showed a gradual enhancement in the compressive and flexural strengths of hybrid fiber-reinforced concrete up to 2.5% lathe waste steel fibers and 0.75% (M5). Further incorporation causes a reduction in strength. The physical examination of fractured samples of hybrid fiber-reinforced concrete confirms that the lathe waste steel fibers yield a maximum strain before breaking down in the concrete matrix. Furthermore, lathe waste steel fibers broke rather than being pulled out, indicating a good bond with the concrete. It is recommended that up to 2.5% lathe waste steel fibers and 0.75% of glass fibers by the total weight of the concrete can be used as hybrid fiber reinforcement for optimum strength achievement.

Macroscopic and microscopic investigations of determining elasto-mechanical properties of limequat fruit.

Given the paramount importance of agricultural products in global health and food security, and the increasing consumer demand, understanding the mechanical behavior of these materials under various conditions is necessary yet challenging. Due to their heterogeneous and non-uniform nature, determining their mechanical behavior is complex. This study employs atomic force microscopy (AFM) to determine the modulus of elasticity of limequat fruit at the microscopic scale and compares it with macroscopic methods. The analyses revealed a statistically significant difference (at the 1% level) in the mechanical behavior determined at the macroscopic scale. The highest modulus of elasticity, 0.752 MPa, was observed using Hertz's theory under complete placement between two parallel planes. The lowest, 0.059 MPa, was noted when a spherical probe compressed a rectangular sample. The average modulus of elasticity of the limequat peel was 2.007 MPa. At the microscopic scale, the modulus of elasticity of the fruit tissue ranged from 0.370 to 0.365 MPa, and for the peel, it was 0.246 MPa. RESEARCH HIGHLIGHTS: Working principles of this innovative technique were elaborated. The AFM technique used provide elasto-mechanical properties determination of cell walls of single living cells extracted from biological materials on the nanoscale. By combining AFM topographical image and nano-indentation of living fruit cells it will be possible to investigate cells' elasto-mechanical properties. Atomic force microscopy holds great potential for monitoring fruit mechanical properties of biological materials.

Food hydroxycinnamic acids alleviate ageing in dermal cells

Dermal damage is inducible by several factors including UV exposure, oxidative stress and inflammation exacerbating skin senescence and degradation of the skin elastic fibers accumulated in ageing accordingly. Which, phenolics of food hydroxycinnamates with a myriad of health benefits are potentially applicable for ageing treatment. Particularly those of food hydroxycinnamic acids, i.e., caffeic, sinapic and rosmarinic acids, that would be efficient against skin ageing. Effectiveness of caffeic, sinapic and rosmarinic acids alleviating ageing was indicated in human dermal fibroblasts (HDF) and co-culture of human keratinocytes (HaCaT) and HDF. Caffeic acid was exhibited as the strongest (p < 0.01) anti-senescent phenolic examined. The studied food hydroxycinnamic acids were shown to induce collagen synthesis in aged HDF with the noted activities inhibiting MMP-1 and IL-6. Their photoaging protections were proved in the co-culture model with significant (p < 0.001) inhibitions against IL-6, IL-8, MMP-1 and MMP-9 (collagen and elastin degrading enzymes). Which, caffeic acid was demonstrated as the most potent photoaging agent among its counterparts. Caffeic, sinapic and rosmarinic acids were proved to be the efficient nutrients for ageing treatment. These functional food hydroxycinnamates are proven on their anti-senescent and photoprotection activities, and capable to maintain homeostasis of dermal cells. Food-derived hydroxycinnamic acids are therefore recommended for innovative product alleviates skin ageing.Graphical

Mapping epidermal and dermal cellular senescence in human skin aging.

Single-cell RNA sequencing and spatial transcriptomics enable unprecedented insight into cellular and molecular pathways implicated in human skin aging and regeneration. Senescent cells are individual cells that are irreversibly cell cycle arrested and can accumulate across the human lifespan due to cell-intrinsic and -extrinsic stressors. With an atlas of single-cell RNA-sequencing and spatial transcriptomics, epidermal and dermal senescence and its effects were investigated, with a focus on melanocytes and fibroblasts. Photoaging due to ultraviolet light exposure was associated with higher burdens of senescent cells, a sign of biological aging, compared to chronological aging. A skin-specific cellular senescence gene set, termed SenSkin™, was curated and confirmed to be elevated in the context of photoaging, chronological aging, and non-replicating CDKN1A+ (p21) cells. In the epidermis, senescent melanocytes were associated with elevated melanin synthesis, suggesting haphazard pigmentation, while in the dermis, senescent reticular dermal fibroblasts were associated with decreased collagen and elastic fiber synthesis. Spatial analysis revealed the tendency for senescent cells to cluster, particularly in photoaged skin. This work proposes a strategy for characterizing age-related skin dysfunction through the lens of cellular senescence and suggests a role for senescent epidermal cells (i.e., melanocytes) and senescent dermal cells (i.e., reticular dermal fibroblasts) in age-related skin sequelae.

Biallelic and monoallelic variants in EFEMP1 can cause a severe and distinct subtype of heritable connective tissue disorder.

Variants in EFEMP1, encoding Fibulin-3, were previously reported as a rare cause of heritable connective tissue disorder (HCTD) with recurrent hernias and joint hypermobility. We report three new cases with biallelic or monoallelic EFEMP1 variants and severe hernia phenotypes. Two male siblings of 10 and 13 years old presented with marfanoid habitus, recurrent inguinal and umbilical hernias, generalized joint hypermobility, and scoliosis. Parents and halfsiblings reported joint hypermobility and umbilical hernias. The eldest boy died at age 16 from incarcerated gastrointestinal herniation complicated by gastric and bowel necrosis with perforation. Autopsy revealed widespread intestinal diverticula. Immunohistochemistry of skin and fascia tissue did not reveal any abnormalities, including normal staining of elastic fibers. Both siblings harbored compound heterozygous likely pathogenic EFEMP1 variants (c.1320 + 2T > A, p.? and c.698G > A, p.Gly233Asp). An unrelated 58-year-old male had marfanoid features, high myopia, recurrent diaphragmatic and inguinal hernias, and chronic gastrointestinal dilatation with severe malabsorption. Both his dizygotic twin-brother and mother had recurrent hernias and high myopia. This man died at 59 years of age, and autopsy showed extensive diaphragmatic herniation, bowel diverticula, and pulmonary emphysema. A heterozygous EFEMP1 splice-variant (c.81 + 1G > A, p.?) was identified, causing exon skipping leading to a start-loss. Targeted genome reanalysis nor RNA-sequencing revealed a second variant at the other allele. The reported individuals expand the clinical and pathological phenotypes of EFEMP1-related disease, a distinct entity within the spectrum of HCTD. The severe and recurrent hernias, gastrointestinal dilatation, and diverticulosis result in an increased risk for life-threatening complications, demanding early recognition and close monitoring.

Topical Platelet Exosomes Reduce Senescence Signaling in Human Skin: An Exploratory Prospective Trial.

Cellular senescence, an irreversible cell cycle arrest with secretory phenotype, is a hallmark of skin aging. Regenerative exosome-based approaches, such as topical human platelet extract (HPE), are emerging to target age-related skin dysfunction. To evaluate the cellular and molecular effects of topical HPE for skin rejuvenation after 12 weeks of twice daily use. Skin biopsies were obtained for histological evaluation of senescence markers, p16INK4a and p21CIP1/WAF1. Telomere-associated foci, coassociation of telomeres, and DNA damage marker, γH2AX, were assessed. RNA sequencing evaluated senescence associated secretory phenotype (SASP) and extracellular matrix pathways. p16INK4a and p21CIP1/WAF1 staining in senescent skin cells revealed low and high expression subgroups that did not correspond to chronological age. Topical HPE significantly reduced high p16INK4a cells in the dermis (p = .02). There was also a decrease in telomere damage after topical HPE (p = .03). In patients with high senescent cells at baseline, there was a 40% reduction in proinflammatory SASP. Extracellular matrix remodeling pathways, including collagen and elastic fibers, were up-regulated. Topical HPE, applied on intact skin, reduced senescence signaling and senescence-associated telomere damage after 12 weeks of twice daily use, targeting a path for skin longevity or healthy skin aging.

Modification of transvaginal polypropylene mesh with co-axis electrospun nanofibrous membrane to alleviate complications following surgical implantation

BackgroundSurgeries for treating pelvic organ prolapse involving the utilization of synthetic mesh have been associated with complications such as mesh erosion, postoperative pain, and dyspareunia. This work aimed to reduce the surgical implantation-associated complications by nanofibrous membranes on the surface of the polypropylene mesh. The nanofiber of the nanofibrous membrane, which was fabricated by co-axial electrospinning, was composed of polyurethane as fiber core and gelatin as the fiber out layer. The biocompatibility of the modified mesh was evaluated in vitro by cell proliferation assay, immunofluorescence stain, hematoxylin-eosin (HE) staining, and mRNA sequencing. Polypropylene mesh and modified mesh were implanted in a rat pelvic organ prolapse model. Mesh-associated complications were documented. HE and Picro-Sirius red staining, immunohistochemistry, and western blotting were conducted to assess the interactions between the modified mesh and vaginal tissues.ResultsThe modified mesh significantly enhanced the proliferation of fibroblasts and exerted a positive regulatory effect on the extracellular matrix anabolism in vitro. When evaluated in vivo, no instances of mesh exposure were observed in the modified mesh group. The modified mesh maintained a relatively stable histological position without penetrating the muscle layer or breaching the epidermis. The collagen content in the vaginal wall of rats with modified mesh was significantly higher, and the collagen I/III ratio was lower, indicating better tissue elasticity. The expression of metalloproteinase was decreased while the expression levels of tissue inhibitor of metalloproteinase were increased in the modified mesh group, suggesting an inhibition of collagen catabolism. The expression of TGF-β1 and the phosphorylation levels of Smad3, p38 and ERK1/2 were significantly increased in the modified mesh group. NM significantly improved the biocompatibility of PP mesh, as evidenced by a reduction in macrophage count, decreased expression levels of TNF-α, and an increase in microvascular density.ConclusionsThe nanofibrous membrane-coated PP mesh effectively reduced the surgical implantation complications by inhibiting the catabolism of collagen in tissues and improving the biocampibility of PP mesh. The incorporation of co-axial fibers composed of polyurethane and gelatin with polypropylene mesh holds promise for the development of enhanced surgical materials for pelvic organ prolapse in clinical applications.

The Role of the Extracellular Matrix in the Pathogenesis and Treatment of Pulmonary Emphysema.

Pulmonary emphysema involves progressive destruction of alveolar walls, leading to enlarged air spaces and impaired gas exchange. While the precise mechanisms responsible for these changes remain unclear, there is growing evidence that the extracellular matrix plays a critical role in the process. An essential feature of pulmonary emphysema is damage to the elastic fiber network surrounding the airspaces, which stores the energy needed to expel air from the lungs. The degradation of these fibers disrupts the mechanical forces involved in respiration, resulting in distension and rupture of alveolar walls. While the initial repair process mainly consists of elastin degradation and resynthesis, continued alveolar wall injury may be associated with increased collagen deposition, resulting in a mixed pattern of emphysema and interstitial fibrosis. Due to the critical role of elastic fiber injury in pulmonary emphysema, preventing damage to this matrix component has emerged as a potential therapeutic strategy. One treatment approach involves the intratracheal administration of hyaluronan, a polysaccharide that prevents elastin breakdown by binding to lung elastic fibers. In clinical trials, inhalation of aerosolized HA decreased elastic fiber injury, as measured by the release of the elastin-specific cross-linking amino acids, desmosine, and isodesmosine. By protecting elastic fibers from enzymatic and oxidative damage, aerosolized HA could alter the natural history of pulmonary emphysema, thereby reducing the risk of respiratory failure.

Hypertension-Induced Biomechanical Modifications in the Aortic Wall and Their Role in Stanford Type B Aortic Dissection.

Hypertension is a major risk factor for the type B aortic dissection (TBAD), while many patients do not manage or regulate their hypertension consistently, leading to stable or unstable hypertension. Currently, the effects of stable and unstable hypertension on the biomechanical properties of the aorta remain unclear. The objective was to identify a blood pressure state that represents a greater risk for TBAD development. A total of 183 samples (108 axial and 75 circumferential) were divided into three groups. Fatigue tensile tests were carried out to simulate normotension, stable hypertension, and unstable hypertension conditions, respectively. Uniaxial tensile tests were performed; thus, the elastic modulus, energy loss, and the peeling force were assessed to evaluate the biomechanical properties. Compared with normal blood pressure, the modulus of elastic fibers decreased under stable hypertension (0.05 ± 0.02 MPa vs. 0.11 ± 0.03 MPa, p < 0.001) and unstable hypertension (0.08 ± 0.02 MPa, p = 0.008), while collagen fibers increased under stable hypertension (2.14 ± 0.51 MPa vs. 1.10 ± 0.24 MPa, p < 0.001) but decreased under unstable hypertension (0.52 ± 0.14 MPa, p < 0.001) in the axial direction. Similar trends were observed circumferentially. Energy loss was highest under unstable hypertension (0.16 ± 0.03 vs. 0.08 ± 0.03, p < 0.001). Peeling force was significantly reduced under stable hypertension (81.69 ± 12.72 N/m vs. 111.10 ± 27.65 N/m, p < 0.001) and further under unstable hypertension (71.37 ± 16.13 N/m, p < 0.001). Stable and unstable hypertension significantly impair the biomechanical properties of the aortic wall, with unstable hypertension leading to greater damage. Hypertensive patients are recommended to strictly follow medical advice to control blood pressure to avoid a higher risk of TBAD due to improper blood pressure management.

Combining ultrasound and elastography for the detection of a non-palpable, non-sonographically visualized Peyronie's plaques. Our experience.

B-mode ultrasound (US) medical imaging is very effective in localizing and describing Peyronie's disease (PD). Moreover, elastography is a new technique used to evaluate tissue elasticity to detect penile Peyronie's plaques that are not visible using standard B-mode US. The main objective of this study was to evaluate the diagnostic efficacy of real-time elastography (RTE) in PD patients and to determine whether its combined use with standard US improved diagnostic accuracy. RTE is also known as strain elastography (SE). Additionally, this study aimed to assess whether RTE was useful for monitoring PD patients undergoing conservative treatment. A group of 37 PD patients in the active phase was selected based on US examination showing isoechoic or hypo-isoechoic plaques, with or without associated hyperechoic or calcified plaque areas. All patients underwent traditional US combined with RTE before starting conservative treatment with antioxidants, during treatmentand after treatment. After each examination with RTE, a specific "Strain Ratio"(SR) was used to identify the specific elasticity of the tissue. Using B-mode US with RTE, we detected all 13 non-palpable penile plaques present in the 37 PD patients (100% of cases). Using only B-mode US, we detected only 8 of the 13 non-palpable plaques (61.5% of cases). The DI of the plaque decreased during and after treatment in all cases, indicating that RTE is effective for monitoring conservative PD treatment. A statistically significant correlation was found between the DI and plaque volume in all patients (p=0.002). Our study has shown that the combination of US and RTE methods allowed for a more accurate diagnosis in PD patients.

Shock wave damage from the ventral side in primary blast injury: An experimental study in pigs

Aim/PurposeThis study aimed to apply a shock wave from the ventral side of a pig and examine its effect to use the results for new body armor production for humans. MethodsSeven male hybrid pigs were used. Each pig was placed under general anesthesia on the experimental table in a blast tube to expose the left lateral position from the front chest area, and shock waves generated by compressed air at 3.0 MPa were applied. We examined changes in vital signs and arterial blood gas in the hyper-acute phase and computed tomography (CT) images, and autopsies were performed for organ damage after 3 h of observation. Pathological examination was performed for lung damage, which is considered a characteristic of shock wave injury. ResultsAll seven pigs survived. Respiratory arrest occurred in two pigs; however, spontaneous breathing resumed promptly afterward. Hypotension occurred at a frequency of 4. No bradycardia or cardiac arrest was observed in any pig. In the arterial blood gas analysis before and immediately after shock wave exposure and 1 hour later, PaO2 decreased immediately but tended to improve thereafter. CT revealed pulmonary contusions and multiple bulla-like lesions on the surface of the lungs. An autopsy showed lung injury in all pigs, particularly in five cases with bulla-like lesions of various sizes on the lung surface across all lobes. Pathological findings showed visceral pleural detachment with elastic fibers from the lung parenchyma, and the cavity lesion on the lung surface comprised bullae. The degree of intra-abdominal hemorrhage varied; however, all but one case showed splenic injury. ConclusionNone of the pigs exposed to shock waves from the ventral side died; however, most showed multiple bullae on the lung surface with lung contusion and splenic injury, which may have been greater than those exposed from the dorsal side. This may be due to the direct impact of the shock wave proceeding from the epigastrium and subcostal region, which are not protected by the skeletal structure of the thorax. These characteristics should be considered when producing new body armor for humans to protect the body from shock waves.

KMnO4/Pb staining allows uranium free imaging of tissue architectures in low vacuum scanning electron microscopy

Scanning electron microscopy under low-vacuum conditions allows high-resolution imaging of complex cell/tissue architectures in nonconductive specimens. However, the conventional methods for metal staining of biological specimens require harmful uranium compounds, which hampers the applications of electron microscopy. Here, we introduce a uranium-free KMnO4/Pb metal staining protocol that allows multiscale imaging of extensive cell/tissue architectures to intensive subcellular ultrastructures. The obtained image contrast was equivalent to that of Ur/Pb staining and sufficient for ultrastructural observation, showing the fine processes of podocytes in the glomerulus, which were invisible by light microscopy. The stainability in the elastic tissue indicated that the distinct histochemical properties of KMnO4 oxidation led to Pb deposition and BSE signal enhancement superior to Ur staining. Elemental analysis clarified that the determinant of the backscattered electron signal intensity was the amount of Pb deposition enhanced by KMnO4 oxidation. This user-friendly method is anticipated to create a new approach for biomedical electron microscopy.

Development of air therapy as a novel therapeutic branch in the field of rehabilitation.

Developing technology in the field of rehabilitation is vital to accelerate recovery and decrease the side effects of current modalities. Rehabilitation is a challenging science in which the main challenge is not just treating the patient but also to shorten the rehabilitation time and avoid harmful effects. Thus, this review demonstrates the possible design and effects of air therapy as a novel treatment branch besides hydrotherapy, electrotherapy, and manual therapy in the field of rehabilitation. The search was conducted over clinical trials, literature reviews, and systematic reviews on the possible effects of treatments that may have similar effects to the newly developed air therapy. This search was conducted in the Web of Science, Scopus, EBSCO, and Medline databases. Air therapy could be used to improve the function of mechanoreceptors, improve circulation and microcirculation, decrease pain, the release of trigger points, regain the elasticity of soft tissues, treat acute and chronic inflammations, decrease muscle cramps and spasticity, strengthen muscle, and decrease muscle fatigue and Decreasing muscle fatigue and delayed muscle soreness. Air therapy is a novel treatment modality that can be used effectively in the field of rehabilitation. Air therapy could be a valuable and safe treatment in rehabilitation.

Age- and sex-specific biomechanics and extracellular matrix remodeling of the ascending aorta in a mouse model of severe Marfan syndrome.

Thoracic aortic aneurysm (TAA) is associated with Marfan syndrome (MFS), a connective tissue disorder caused by mutations in fibrillin-1. Sexual dimorphism has been recorded for TAA outcomes in MFS, but detailed studies on the differences in TAA progression in males and females and their relationships to outcomes have not been performed. The aims of this study were to determine sex differences in the diameter dilatation, mechanical properties, and extracellular matrix (ECM) remodeling over time in a severe mouse model (Fbn1mgR/mgR = MU) of MFS-associated TAA that has a shortened life span. Male and female MU and wildtype (WT) mice were used at 1-4 mo of age. Blood pressure and in vivo diameters of the ascending thoracic aorta were recorded using a tail-cuff system and ultrasound imaging, respectively. Ex vivo mechanics and ECM remodeling of the aorta were characterized using a biaxial test system and multiphoton imaging, respectively. We showed that mechanical properties, such as structural and material stiffness, and ECM remodeling, such as elastic and collagen fiber content, correlated with diameter dilatation during TAA progression. Male MU mice had accelerated rates of diameter dilatation, stiffening, and ECM remodeling compared with female MU mice which may have contributed to their decreased life span. The correlation of mechanical properties and ECM remodeling with diameter dilatation suggests that they may be useful biomarkers for TAA progression. The differences in diameter dilatation and life spans in male and female MU mice indicate that sex is an important consideration for managing thoracic aortic aneurysm in MFS. NEW & NOTEWORTHY Using a mouse model (Fbn1mgR/mgR = MU) of severe thoracic aortic aneurysm in Marfan syndrome (MFS), we found that male MU aorta had an accelerated time line and increased amounts of dilatation, stiffening, and extracellular matrix (ECM) remodeling compared with female MU aorta that may have contributed to an increased risk of fatigue failure with cyclic loading over time and a reduced life span. We suggest that aortic stiffness may provide useful information for clinical management of aneurysms in MFS.

Amine-impregnated elastic carbon nanofiber aerogel templated by bacterial cellulose for CO2 adsorption and separation

Carbon aerogel has become a promising electrode material for CO2 capture and capacitor due to its large specific surface area, well-developed pore structure, adjustable porosity and ultra-high specific power. However, most of the carbon-based materials suffer from poor mechanical properties, low recycling efficiency, and low adsorption capacity, making it difficult to be put into industrial applications on a large scale. Bacterial cellulose (BC) has an ultra-fine reticular structure, a modulus of elasticity that is several to more than ten times that of typical plant fibers, and high tensile strength. In this paper, BC was treated by unidirectional freeze-drying method, and the aerogels with ordered honeycomb pore structure were constructed by unidirectional growth of ice crystals, and the pyrolytic chemical properties of BC were adjusted by using (NH4)2SO4, which effectively prevented the shrinkage and deformation of materials during carbonization, and successfully prepared elastic nanocarbon fiber aerogels. At the same time, the introduction of (NH4)2SO4 made the material realize N doping in the carbonization process, providing more adsorption sites, which was conducive to the adsorption of CO2. Finally, the carbon fiber nanoaerogel was modified by TEPA to further improve its adsorption selectivity for CO2. The prepared elastic carbon nanofiber aerogel (CBCNT) has high CO2 adsorption performance and high selectivity, and its CO2 capture capacity is up to 4.88 mmol/g. At the same time, its capacitance also reached 246F/g.

The first Japanese case of autosomal dominant cutis laxa with a frameshift mutation in exon 30 of the elastin gene complicated by small airway disease with 8 years of follow-up

BackgroundCutis laxa constitutes a diverse group of connective tissue diseases, both inherited and acquired, characterized by loose skin and varying systemic involvement, including pulmonary lesions. While cutis laxa has been linked to conditions like emphysema, asthma, and bronchiectasis, the specific pathological and radiological characteristics underlying pulmonary complications related to cutis laxa remain unclear.Case presentationA 36-year-old woman, diagnosed with cutis laxa at birth, presented to our outpatient clinic with severe obstructive ventilatory impairment, evident in pulmonary function tests (expiratory volume in one second (FEV1)/forced vital capacity (FVC): 34.85%; %residual volume [RV]: 186.5%; %total lung capacity [TLC]: 129.2%). Pulmonary function tests also indicated small airway disease (%FEF50%, 7.9%; %FEF75%, 5.7%; and %FEF25–75%, 6.8%). Computed tomography (CT) revealed the lack of normal increase in lung attenuation on expiratory CT scan, with no discernible emphysematous changes. Exome sequencing was performed to confirm the association between the pulmonary lesions and cutis laxa, revealing a frameshift variant in exon 30 of the elastin gene (ELN). Further analysis employing a parametric response map revealed a longitudinal increase in the percentage of functional small airway disease (fSAD) from 37.84% to 46.61% over the 8-year follow-up, despite the absence of overt changes in CT findings, specifically the lack of normal increase in lung attenuation on expiratory CT scan. Over the same follow-up interval, there was a modest reduction of 25.6 mL/year in FEV1 coupled with a significant increase in %RV. Pulmonary function test metrics, reflective of small airway disease, exhibited a continual decline; specifically, %FEF50%, %FEF75%, and %FEF25–75% diminished from 7.9% to 7.0%, 5.7% to 4.6%, and 6.8% to 5.4%, respectively.ConclusionsThis case highlighted an instance of autosomal dominant cutis laxa arising from a frameshift variant in exon 30 of ELN, accompanied by small airway disease. Comprehensive investigation, utilizing quantitative CT analysis, revealed a longitudinal increase in fSAD percentage with a mild reduction in FEV1. These findings indicate that elastin deficiency may not only diminish elastic fibers in the skin but also be implicated in small airway disease by impacting components of the extracellular matrix in the lungs.

Interplay of atherosclerosis and medial degeneration in human ascending aorta

The previous understanding has been that atherosclerosis tends to increase distally from the ascending aorta, but recent studies and practical experience have indicated that atherosclerosis occurs in the ascending aorta more than previously thought. Medial degeneration is linked to aortic aneurysms, dissection and dilatation and has been related to increased mortality. There is a lack of data on the coexistence of atherosclerosis and medial degeneration in the ascending aorta and its outcome to clinical morbidity and mortality. Earlier studies have shown coexisting atherosclerosis and medial degeneration as significant risk indicators for coronary and cerebrovascular events. We aimed to analyze aortic specimens classified according to the consensus documents of the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology particularly the comparison of variable morphological features with the atherosclerotic grade to gain more data about the coexistence of atherosclerosis and medial degeneration. We evaluated 217 specimens of human ascending aorta resected at Tampere University Heart Hospital because of aortic aneurysm, dissection or dilatation. None of the samples contained normal aortic morphology; atherosclerosis was found in a total of 75.8% of the samples and medial degeneration in all the samples. The present study is mostly in agreement with earlier research regarding the prevalence of different histological findings, even though a higher prevalence of atherosclerosis was found compared with most studies. There was no statistically significant association between atherosclerosis and medial degeneration, but a higher atherosclerotic grade was significantly associated with the presence of smooth muscle cell nuclei loss, smooth muscle cell disorganisation, elastic fibre thinning and medial fibrosis. Our study reinforces the perception that atherosclerotic lesions significantly occur in the ascending aorta and coexist with individual components of the medial degeneration.

Histological characterization of rat vocal fold across different postnatal periods.

To evaluate the vocal fold histological characteristics during different postnatal periods in rats, especially older rats. Sprague-Dawley rats aged 4 days, 4 and 12 weeks, and 12 and 24 months were used for the experiment. Five larynges were obtained for each age and cut into 5-μm consecutive sections. The expression of Ki-67 was assessed using immunohistochemistry to examine cell proliferation. Elastic van Gieson staining was used to detect the collagen and elastin concentrations. The cell type was determined using multicolor immunofluorescence. Ki-67 was not expressed in the macula flava (MF) of 12-week-, 12-month-, and 24-month-old adults. Collagen fibers in the lamina propria (LP) increased with age. The elastic fiber concentrations in the LP decreased significantly at 24 months (p < .01) but remained stable in the MF. All posterior MF cells showed strong glial fibrillary acidic protein and vimentin-positive reactions with weaker expressions of CD68 and α-smooth muscle actin (α-SMA). The myofibroblasts (α-SMA-positive) and macrophages (CD68-positive) in the LP of the 24-month-old rats were significantly the highest (p < .01). The extracellular matrix in the LP increases with age, presenting as an increase in collagen with the loss of elastin, which may be due to myofibroblast proliferation. Moreover, the cellular properties or extracellular matrix components of the mature MF in rats are comparable to those in humans.