• Some carriers are unaffected, while others develop a less severe condition called thin basement membrane nephropathy, which is characterized by hematuria. (medlineplus.gov)
  • It remains unclear why some individuals with one variant in the COL4A3 or COL4A4 gene have progressive kidney disease (autosomal dominant Alport syndrome) and others have only hematuria (thin basement membrane nephropathy). (medlineplus.gov)
  • Objective -To evaluate expression of the α6 chain of type IV collagen in the glomerular basement membranes (GBM) of healthy dogs. (avma.org)
  • Alport syndrome is a nephritic syndrome caused by a mutation in the COL4A3 , COL4A4 , and COL4A5 genes that encode the alpha-5 chain of type IV collagen and results in altered type IV collagen strands. (msdmanuals.com)
  • After analysis of deduced amino acid sequences from cloned cDNAs of dipeptidyl peptidase IV (DPP IV) and aminopeptidase A (APA), it was noticed that DPP IV and APA are type II membrane proteins that have one transmembrane domain near the N-terminus. (nature.com)
  • The GRS brings together early career researchers involved in diverse areas of collagen research and provides a supportive and stimulating environment to share and discuss emerging research on collagen proteins. (grc.org)
  • Our bodies create collagen by breaking down the protein we eat into amino acids , which are the building blocks from which our bodies can form new proteins. (webmd.com)
  • Peptides are compounds composed of amino acids, which form the building blocks of proteins in our skin, including collagen. (lifeextension.com)
  • Further study applying proteomic analysis confirmed that CAFs secrete more proteases, extra-cellular matrix proteins, and proteins that modify the basement membrane compared with NAFs, pointing to a remodeling role for CAFs in invasion. (medindia.net)
  • Because type IV collagen and laminin are proteins found exclusively in the basement membrane, the antibodies to these two proteins selectively and specifically recognize basement membrane components. (biogenex.com)
  • Collagen alpha-4(IV) chain is a protein that in humans is encoded by the COL4A4 gene. (wikipedia.org)
  • In addition, defects in processing chondrocalcin, a calcium binding protein that is the C-propeptide of this collagen molecule, are also associated with chondrodysplasia. (nih.gov)
  • The proteolytically produced C-terminal fragment of type XV collagen is restin, a potentially antiangiogenic protein that is closely related to endostatin. (nih.gov)
  • Amino acid sequence of mouse nidogen, a multidomain basement membrane protein with binding activity for laminin, collagen IV and cells. (unifi.it)
  • To improve the comprehensibility of electron micrographs, endothelial cells (E) have been transparently highlighted in BD-3 Protein Rat yellow, though basement membranes were transparently highlighted in red. (c-mycinhibitor.com)
  • ECM was prepared to a protein concentration of 8-12 mg/mL (in DMEM), containing laminin as a major component, collagen type IV, heparin sulfate proteoglycan, entactin, and other minor components. (sigmaaldrich.com)
  • These genes each provide instructions for making one component of a protein called collagen IV. (medlineplus.gov)
  • Kidneys with abnormal collagen IV cannot properly filter the blood, which allows blood and protein to pass into the urine, causing hematuria and proteinuria. (medlineplus.gov)
  • Collagen is a protein that is essential to the health and function of connective tissues in your body. (webmd.com)
  • Lastly, we revealed that addition of type IV collagen protein induced myofibroblast proliferation and migration in monolayer culture and increased the formation of mesenchymal-epithelial septal-like structures in co-culture. (biomedcentral.com)
  • d - i Type IV collagen protein and mRNA expression in the lung. (biomedcentral.com)
  • Collagen is the main protein of connective tissues in humans and accounts for 70% - 80% of the skin's dry weight. (lifeextension.com)
  • Fibroblasts are most commonly found in connective tissue that synthesizes extra-cellular matrix and another main structural protein of animals, collagen. (medindia.net)
  • Two compounds, Compound 1 (1,4-dihydrophenanthrolin-4-one-3-carboxylic acid) and Compound 5 [8-(N-butyl-N-ethylcarbamoyl)-1,4-dihydrophenathrolin-4-one-3-carboxylic acid], with comparable potencies in vivo, were chosen to investigate the effect of the inhibition of the hydroxylation of newly synthesized uterine collagen on the turnover of this protein in vivo. (embl.de)
  • Taken with the present results, this suggests that the potential use of inhibitors of prolyl 4-hydroxylase to control excessive collagen deposition in pathological fibrosis may be limited by the need to maintain continuous inhibition of collagen hydroxylation so as to facilitate intracellular degradation of the accumulated protein. (embl.de)
  • It was also found that visfatin upregulated collagen I and III production, procollagen I and III mRNA expression and protein production. (thieme-connect.com)
  • We conclude that the stability of the helical structure of type IV collagen within a basement membrane is considerably greater than it is in solution, and that conformation-dependent monoclonal antibodies can be useful probes for investigations of molecular structure in situ. (rupress.org)
  • COPD airways had more vessels (with greater permeability) in the Rbm (P = 0.01) and fewer vessels (with normal permeability) in the LP compared to controls with both Collagen IV and Factor VIII antibodies (P = 0.04 and P = 0.01). (edu.au)
  • Immunohistochemical staining was performed using antibodies against the major components of basement membranes such as laminin, heparan sulfate proteoglycan (HSPG), Collagen IV and fibronectin. (thieme-connect.com)
  • Capture antibodies are supplied arrayed/spotted on a membrane with each pair of spots representing a different analyte. (abcam.com)
  • Sample is added (0.2-1ml of 1 sample to each membrane), and then paired biotinylated detector antibodies and streptavidin HRP. (abcam.com)
  • Because, in IDDM patients with albuminuria, alterations of the content of HS and collagen IV within the capillary BM have been demonstrated immunohistochemically, not only in the glomerular filtration barrier, but also in the skeletal muscle capillary BM, we suggest that these changes reflect universal quantitative or qualitative alterations within the capillary filtration barrier. (diabetesjournals.org)
  • Objective -To determine whether an alteration in calcium regulation by skeletal muscle sarcoplasmic reticulum, similar to known defects that cause malignant hyperthermia (MH), could be identified in membrane vesicles isolated from the muscles of Thoroughbreds with recurrent exertional rhabdomyolysis (RER). (avma.org)
  • Skeletal muscle membrane vesicles, prepared by differential centrifugation of muscle tissue homogenates obtained from the horses, were characterized for sarcoplasmic reticulum (SR) activities, including the Ca 2+ release rate for the ryanodine receptor-Ca 2+ release channel, [ 3 H]ryanodine binding activities, and rate of SR Ca 2+ -ATPase activity and its activation by Ca 2+ . (avma.org)
  • Sarcolemma of skeletal muscle prepared by a quick-freeze deep-etch method also shows the basement membrane (lamina externa, labeled here as the basal lamina) associated with the muscle fiber. (cellimagelibrary.org)
  • This gene encodes the alpha-1 chain of type II collagen, a fibrillar collagen found in cartilage and the vitreous humor of the eye. (nih.gov)
  • Fibrillar collagens (type I and III) reside in the dermis where they form well-organized, rope-like fibers that supply robust tensile strength and resistance to stretching and tearing. (lifeextension.com)
  • Non-fibrillar collagens (type IV, VII, and XVII) reside in the dermo-epidermal junction where they self-assemble a scaffolding network that anchors the dermis to the epidermis above it. (lifeextension.com)
  • Decreased production of non-fibrillar collagens also contributes to skin aging. (lifeextension.com)
  • 9 This enzyme, together with the collagenase produced by neutrophils and classified as MMP-8, are the only mammalian enzymes known to be capable of initiating hydrolysis of fibrillar type I collagen, 2 10 the major component of the corneal stroma. (bmj.com)
  • Binding sites for laminin and collagen IV are present on the C-terminal globule but not yet precisely localized. (unifi.it)
  • Composition and structural organization of type IV collagen in the GBM of healthy adult dogs is different from that described for other species. (avma.org)
  • Thermal stability of the helical structure of type IV collagen within basement membranes in situ: determination with a conformation-dependent monoclonal antibody. (rupress.org)
  • To examine the thermal stability of the helical structure of type IV collagen within basement membranes in situ, we have employed indirect immunofluorescence histochemistry performed at progressively higher temperatures using a conformation-dependent antibody, IV-IA8. (rupress.org)
  • This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. (wikipedia.org)
  • Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. (wikipedia.org)
  • Mutations in this gene are associated with type II autosomal recessive Alport syndrome (hereditary glomerulonephropathy) and with familial benign hematuria (thin basement membrane disease). (wikipedia.org)
  • Type IV collagen: structure, gene organization, and role in human diseases. (wikipedia.org)
  • This gene encodes the alpha chain of type XV collagen, a member of the FACIT collagen family (fibril-associated collagens with interrupted helices). (nih.gov)
  • Type XV collagen has a wide tissue distribution but the strongest expression is localized to basement membrane zones so it may function to adhere basement membranes to underlying connective tissue stroma. (nih.gov)
  • Addition of collagen type IV to the ECM gel increases polymerization. (sigmaaldrich.com)
  • Basement membranes were stained with anti-type IV collagen. (nih.gov)
  • Alport's syndrome, Goodpasture's syndrome, and type IV collagen. (nih.gov)
  • Type I collagen is responsible for giving your skin a plump, youthful appearance. (webmd.com)
  • Type IV collagen is the main component of the basement membrane that gives strength to the blood-gas barrier (BGB). (biomedcentral.com)
  • however, the specific role of type IV collagen in regulating alveologenesis remains unknown. (biomedcentral.com)
  • Our study showed that type IV collagen and, therefore the basement membrane, play fundamental roles in coordinating alveolar morphogenesis. (biomedcentral.com)
  • In addition to its role in the formation of epithelium and vasculature, type IV collagen appears to be key for alveolar myofibroblast development by inducing their proliferation, differentiation and migration throughout the developing septum. (biomedcentral.com)
  • Lung development timeline and type IV collagen expression in the chicken and the mouse. (biomedcentral.com)
  • Many commercial topical creams target type I collagen . (lifeextension.com)
  • 4 In fact, type I collagen is stronger than steel on a gram-for-gram basis. (lifeextension.com)
  • Many topical products focus only on type I collagen without recognizing the significance of other collagen types in restoring youthful skin integrity and function. (lifeextension.com)
  • This gene encodes the major type IV alpha collagen chain of basement membranes. (antibodies-online.com)
  • Laminin and type IV collagen are the two principal components of the basement membrane. (biogenex.com)
  • The gelatinases MMP-2 and MMP-9 are enzymes that will hydrolyse type IV collagen, the major component of basement membranes. (bmj.com)
  • Enzyme-linked immunosorbent assay was used for measuring collagen type I and III production. (thieme-connect.com)
  • Cause is a gene mutation affecting type IV collagen. (msdmanuals.com)
  • They are classified into 5 groups according to substrate specificity and internal homology: collagenases, gelatinases, stromelysins, membrane-type MMPs, and others, including matrilysins 8 . (bvsalud.org)
  • In a published international consensus statement on mucous membrane pemphigoid, an expert panel decided to include this group of patients (previously designated as epidermolysis bullosa acquisita based in part on their autoantibodies to type VII collagen) in the category of mucous membrane pemphigoid. (medscape.com)
  • The granulomas are arranged in a tierlike (layered) fashion and are admixed with areas of collagen degeneration. (medscape.com)
  • Anti-Collagen IV antibody detected more vessels in the Rbm (P = 0.002) and larger vessels in both Rbm (P (edu.au)
  • Cytokine arrays are an antibody-pair-based assay, analogous to ELISA, but using a membrane as a substrate rather than a plate. (abcam.com)
  • A table listing all of our human membrane antibody cytokine arrays and other arrays and the analytes they measure is available here . (abcam.com)
  • Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production. (medscape.com)
  • An analysis of the basement membrane, which can modulate epithelial proliferation, reveals a dysfunction of this layer between cholesteatoma and stroma. (thieme-connect.com)
  • Visfatin is a novel adipokine involved in the regulation of inflammatory cytokines, however, the effects of visfatin on proliferation and collagen synthesis of CFs are unclear. (thieme-connect.com)
  • The aim of this study was to determine whether visfatin has any effect on the proliferation and collagen synthesis in rat CFs. (thieme-connect.com)
  • These results suggest that visfatin promote CFs proliferation and collagen synthesis via p38MAPK, PI3K, and ERK 1/2 pathways rather than JNK pathways, which also indicate that visfatin might play a role in myocardial fibrosis. (thieme-connect.com)
  • During an inflammatory response, neutrophils move from the blood to the site of inflammation by transmigrating across the endothelial barrier and through the basement membrane (BM) of the surrounding extracellular matrix (ECM). (nature.com)
  • One potential limit to the efficacy of intravitreal viral injection stems from the physical barriers formed by the vitreous, internal limiting membrane (ILM), retinal extracellular matrix (ECM), and cell surface proteoglycans. (molvis.org)
  • The enzyme responsible for collagen maturation is prolyl 4-hydroxylase, making this enzyme a central activity in cuticle biosynthesis and a potentially important chemotherapeutic target. (embl.de)
  • Examples of a novel series of phenanthrolinones are shown to be potent competitive inhibitors of avian prolyl 4-hydroxylase, and of collagen hydroxylation, in embryonic chick tendon cells and human foreskin fibroblasts in vitro and in the oestradiol-stimulated rat uterus in vivo. (embl.de)
  • Earlier reports indicated that underhydroxylated collagen, accumulating within the endoplasmic reticulum in cells where prolyl 4-hydroxylase is inactivated, is slowly degraded, but is then rapidly hydroxylated and secreted when the activity of prolyl 4-hydroxylase is restored. (embl.de)
  • In a subset of patients with predominant mucous membrane involvement, the disease manifests with blisters and scar formation in the oral, ocular, vaginal, and other mucous membranes, leading to significant dysfunction, such as visual function loss, dysphagia, malnutrition, or even mortality. (medscape.com)
  • The clinical phenotype of this subset of patients is indistinguishable from that of mucous membrane pemphigoid. (medscape.com)
  • The rationale for such inclusion is that this subset of patients has the clinical phenotype of mucous membrane pemphigoid and that the autoantibodies of patients with mucous membrane pemphigoid (as a group) target not a single, but multiple, skin basement membrane components, such as bullous pemphigoid antigen 2 (BP180), integrin beta-4 subunit, laminin-5, and laminin-6. (medscape.com)
  • Because this subset of patients cannot be distinguished from mucous membrane pemphigoid by clinical phenotype and autoantigen identity alone cannot be used to include or exclude a diagnosis of mucous membrane pemphigoid, it seems reasonable to include this subset of patients under the general category of mucous membrane pemphigoid. (medscape.com)
  • The skin and mucous membrane manifestations of epidermolysis bullosa acquisita (EBA) take several forms, including a noninflammatory or mildly inflammatory disease, a generalized inflammatory disease, and a predominant mucous membrane disease. (medscape.com)
  • This variant clinically resembles mucous membrane pemphigoid and can result in significant mucosal scarring and dysfunction. (medscape.com)
  • An international consensus statement published in Archives of Dermatology (March 2002) reassigned this group of patients to the category of mucous membrane pemphigoid. (medscape.com)
  • The results indicate that emission from dental light curing units can affect the oral mucous membrane and may reduce its functional abilities. (who.int)
  • Standing between a cancer cell in situ and the surrounding tissue of fibroblasts and extra-cellular matrix is the basement membrane, a thin sheet of fibers that normally cradles the cells above it. (medindia.net)
  • But there are also carcinoma-associated fibroblasts (CAF) whose matrix proteinases could come in handy in breaking the membrane barrier. (medindia.net)
  • do cancer cells invade the basement membrane or do some fibroblasts help invading cancers? (medindia.net)
  • Using human colon cancer cells and primary human fibroblasts isolated from tumors and adjacent normal tissues, Alexandros Glentis and colleagues at the Institut Curie addressed the question of whether the cancer cells or the CAF cells were responsible for the breakdown of the basement membrane that leads to cancer progression. (medindia.net)
  • The researchers then devised an assay that deployed native basement membrane to separate cancer cells on one side and fibroblasts embedded in collagen on the other. (medindia.net)
  • Through imaging the tumors and fibroblasts, the researchers then found the smoking gun of cancer-CAF cooperation--long protrusions like puppet-strings that the tumor cells used to communicate with the fibroblasts well before the cancer cells moved to breach the basement membrane. (medindia.net)
  • Aging decreases the synthesis of collagen fibers in the dermis, and causes existing collagen fibers to become disorganized, stiff, and fragmented. (lifeextension.com)
  • That alterations of collagen VII result in blister formation, scar deficiency associated with inflammation, and increased expression of transforming growth factor β. (thieme-connect.de)
  • Microhematuria and subsequently proteinuria are hallmarks of kidney involvement, which are due to primary basement membrane alterations that mainly cause endothelial thrombosis and podocyte contraction and ulterior irreversible detachment. (hindawi.com)
  • These environmental factors generate inflammation and oxidative stress that trigger activation of collagen-destroying enzymes known as matrix metalloproteinases (MMPs). (lifeextension.com)
  • Recessive dystrophic epidermolysis bullosa (RDEB) is a severe subtype of epidermolysis bullosa caused by changes in collagen VII with a high risk of early development of cutaneous squamous cell carcinoma (cSCC). (thieme-connect.de)
  • A third variant of epidermolysis bullosa acquisita predominantly involves mucous membranes. (medscape.com)
  • Research indicates that taking collagen peptide supplements can increase bone mineral density in women who have gone through menopause . (webmd.com)
  • Collagen peptide supplementation in combination with resistance training improves body composition and increases muscle strength in elderly sarcopenic men: a randomised controlled trial. (webmd.com)
  • Renin-angiotensin system and myocardial fibrosis in hypertension: regulation of the myocardial collagen matrix. (thieme-connect.com)
  • Crystal structure of the human collagen XV trimerization domain: a potent trimerizing unit common to multiplexin collagens. (nih.gov)
  • Control mice (C57BL/6J Ola/Hsd strain) or mice lacking either collagen XV (Col15a1−/−) or XVIII (Col18a1−/−) were included in the study. (uib.no)
  • Necrobiosis lipoidica is a disorder of collagen degeneration with a granulomatous response, thickening of blood vessel walls, and fat deposition. (medscape.com)
  • This image shows the heterogeneity in the content of lysosomes (Lys) near the plasma membrane between two hepatocytes and near the bile canaliculus (not visible in this field). (cellimagelibrary.org)
  • AGE-modified collagen in gingival blood vessel walls binds circulating LDL, which is frequently elevated in diabetes, resulting in atheroma formation and further narrowing of the vessel lumen. (health.am)
  • Human umbilical vein endothelial cells formed confluent vessels against collagen I and geltrex mix, a mix of basement membrane extract and collagen I. TNF-α-stimulation of vessels upregulated inflammatory cytokine expression and promoted neutrophil transmigration. (nature.com)
  • The focus of the 2023 Collagen GRS is to highlight interdisciplinary topics in collagen research related, but not limited to, the broad range of physiological processes collagens are involved in and how this knowledge can aid in our understanding of collagen molecular properties, and the development of novel treatments and therapies. (grc.org)
  • All attendees of the 2023 Collagen GRS are encouraged to attend the subsequent 2023 Gordon Research Conference (GRC) on Collagen. (grc.org)
  • We utilized N- and C-terminal split NanoLuciferase assays to determine the effect of a novel COL4A4 variant of uncertain significance (VUS) on collagen IV heterotrimer formation in vitro. (stanford.edu)
  • A group of peptides has been shown to activate growth factors that promote youthful collagen synthesis . (lifeextension.com)
  • Incorporation of [ 3 H]-thymidine and [ 3 H]-proline were used for evaluating DNA and collagen synthesis. (thieme-connect.com)
  • We demonstrated that visfatin significantly increased DNA and collagen synthesis in a dose- and time-dependent manner. (thieme-connect.com)
  • Tumor suppression by collagen XV is independent of the restin domain. (nih.gov)
  • Two transcripts, differing only in their transcription start sites, have been identified for this gene and, as is common for collagen genes, multiple polyadenylation sites are found in the 3' UTR. (wikipedia.org)
  • We aimed to study the physiological roles of these two components of the permeability barrier non-invasively in striated muscle in mice deficient in collagen XV or XVIII by dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). (uib.no)
  • The researchers are currently testing a role of CAF-derived molecules in basement membrane remodeling to further dissect the secret alliance between cancer cells and CAFs in basement membrane invasion. (medindia.net)
  • The cuticle of parasitic nematodes consists primarily of a network of collagen molecules. (embl.de)
  • Mouse studies have shown that collagen XV deficiency is associated with muscle and microvessel deterioration. (nih.gov)
  • For older people experiencing muscle deterioration, collagen may help strengthen muscles. (webmd.com)
  • This staining pattern resembles the initial rear-rangement of the epithelial basement membrane in human skin wounds and provides evidence for a lost contact inhibition on the provisional fibronectin-layer as a reason for the hyperproliferative nature of cholesteatoma lesions. (thieme-connect.com)
  • The formation of AGEs results in collagen accumulation in the periodontal capillary basement membranes, causing membrane thickening. (health.am)
  • Cell damage was assessed using MTT assays, and the lactate dehydrogenase (LDH) release rate and flow cytometry were used to detect the accumulation of reactive oxygen species (ROS) and mitochondrial membrane potential respectively. (sdbonline.org)
  • Both collagen XV and XVIII are important for maintaining normal capillary permeability in the striated muscle. (uib.no)
  • Under these conditions, the in situ denaturation temperature of this epitope in most basement membranes is 50-55 degrees C. In capillaries and some other small blood vessels the fluorescent signal is still clearly detectable at 60 degrees C, the highest temperature at which we can confidently use this technique. (rupress.org)
  • This study compared Collagen IV staining with vessels marked with anti-Factor VIII and examined vessel permeability in bronchial biopsies from COPD and normal subjects using albumin staining. (edu.au)
  • a) double immunofluorescence labeling in sections from 4 h pMCAO animals showing the distribution in the Cx43related immunosignals and collagen IV, whereas the latter of which demarks cerebral vessels. (c-mycinhibitor.com)
  • Inhibition of neutrophil proteases inhibited neutrophil transmigration on geltrex mix, but not collagen I. These findings highlight the important role of the ECM in determining cell phenotype and response to inhibitors. (nature.com)
  • GSOs also mitigated the deleterious effects of GLU on the mitochondrial membrane potential and Cyt C release, thus alleviating mitochondrial dysfunction, and increased GSH levels and CAT activity in both cells and Drosophila brain tissue. (sdbonline.org)
  • Variants in these genes result in the production of abnormal versions of collagen IV in glomeruli. (medlineplus.gov)
  • Abnormal versions of collagen IV in the eye can result in misshapen lenses and an abnormally colored retina. (medlineplus.gov)
  • A topical formula with a trio of peptides has been shown to synthesize all major collagen types to revitalize aging skin. (lifeextension.com)
  • A topical formula featuring three peptides has been shown to synthesize all major collagen types in the dermis and dermo-epidermal junction. (lifeextension.com)
  • That is why scientists developed three unique peptides that renew all major collagen types to rejuvenate aged and damaged skin. (lifeextension.com)