• Objective -To evaluate expression of the α6 chain of type IV collagen in the glomerular basement membranes (GBM) of healthy dogs. (avma.org)
  • Glomerular basement membrane. (wikipedia.org)
  • No specific or efficient treatment exists for Alport syndrome, an X-linked hereditary disease caused by mutations in collagen type IV, a crucial component of the glomerular basement membrane. (hindawi.com)
  • Urinary podocyte loss is a silent phenomenon that precedes proteinuria in glomerular diseases [ 3 , 4 ]. (hindawi.com)
  • Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating anti-glomerular basement membrane (anti-GBM) antibodies. (medscape.com)
  • Anti-GBM disease is an autoimmune disorder characterized by autoantibodies directed against the glomerular/alveolar basement membrane. (medscape.com)
  • This binding of antibodies can be visualized as the linear deposition of immunoglobulin along the glomerular basement membrane and, less commonly, the alveolar basement membranes, by direct immunofluorescent techniques. (medscape.com)
  • The α-5 (IV) chain of collagen is 1,685 amino acids in length and is expressed at 225 locations in the human glomerular basement membrane, retinal pigment layer, main visual cortex, and cochlea. (researchsquare.com)
  • The glomerular basement membrane (GBM) is an important component of the kidney's glomerular filtration barrier. (wustl.edu)
  • Funk, SD , Lin, MH & Miner, JH 2018, ' Alport syndrome and Pierson syndrome: Diseases of the glomerular basement membrane ', Matrix Biology , vol. 71-72, pp. 250-261. (wustl.edu)
  • in most families, thickening and thinning of the glomerular and tubular basement membranes occur, with multilamination of the lamina densa in a focal or local distribution (basket-weave pattern). (msdmanuals.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)
  • 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)
  • The antiglomerular basement membrane antibodies were strongly positive. (medscape.com)
  • In the present study, ultrastructural immunolocalization with monoclonal and monospecific polyclonal antibodies to type VII collagen and with a monoclonal antibody to type IV collagen indicates that amorphous electron-dense structures which we term 'anchoring plaques' are normal features of the basement membrane zone of skin and cornea. (elsevierpure.com)
  • [ 4 ] confirmed that the antibodies taken from the diseased kidneys produced nephritis in experimental animals. (medscape.com)
  • [ 5 , 6 ] Two conformational epitopes of anti-GBM antibodies have been defined at residues 17-31 and 127-141 of alpha3(IV)NC1, which were named as EA and EB, respectively. (medscape.com)
  • Collagen alpha-4(IV) chain is a protein that in humans is encoded by the COL4A4 gene. (wikipedia.org)
  • SVEVs isolated from lyophilized venoms collected from four different species of snakes ( Agkistrodon contortrix contortrix , Crotalus atrox , Crotalus viridis and Crotalus cerberus oreganus ) were analyzed by mass spectrometry-based proteomic, which allowed the identification of proteins belonging to eight main functional protein classes such as SVMPs, serine proteinases, PLA 2 , LAAO, 5′nucleotidase, C-type lectin, CRISP and Disintegrin. (nature.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)
  • 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)
  • 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)
  • It was also found that visfatin upregulated collagen I and III production, procollagen I and III mRNA expression and protein production. (thieme-connect.com)
  • TGF-β2 is capable of inducing the expression of ECM and basement membrane components in cultured ONH astrocytes via CTGF and upregulated TSP-1, a protein naturally involved in the activation of latent TGF-β. (arvojournals.org)
  • This latter effect was due to protein kinase C activation in that it was inhibited by sphingosine and was not seen with 4 alpha-PMA, an inactive analogue. (jci.org)
  • Abnormal type IV collagen in glomeruli can prevent the kidneys from adequately filtering the blood and allows blood and protein to pass into the urine, resulting in progressive scarring of kidney tissue [2] . (rbht.nhs.uk)
  • The most abundant protein to be found in the human body, collagen for skin has endless health benefits and deserves to be a part of your beauty routine. (femina.in)
  • Collagen is a protein found throughout the body that provides structure and support to tissues such as skin, bone, and cartilage. (femina.in)
  • Collagen is a protein that is naturally found in our bodies and is responsible for maintaining the structure and elasticity of our skin. (femina.in)
  • The predominant form of the protein is comprised of two alpha1(IV) subunits and one alpha2(IV) subunit, however, at least six different alpha subunits can be incorporated into the heterotrimer. (uchicago.edu)
  • About one quarter of all of the protein in your body is collagen. (rcsb.org)
  • Collagen is a major structural protein, forming molecular cables that strengthen the tendons and resilient sheets that support the skin and internal organs. (rcsb.org)
  • But, in spite of its critical function in the body, collagen is a relatively simple protein. (rcsb.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)
  • 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)
  • Alport syndrome is an inherited disorder of the basement membrane, resulting in progressive renal failure due to glomerulonephropathy, variable sensorineural hearing loss, and variable ocular anomalies [1] . (rbht.nhs.uk)
  • X-linked Alport syndrome is largely caused by mutations in COL4A5 which codes for a component of type IV collagen. (rbht.nhs.uk)
  • Homozygote Mutationen in dem Typ IV Kollagen (COL4A3, COL4A4 und COL4A5) führen zum Alport-Syndrom. (uni-goettingen.de)
  • Heterozygote Mutationen führen zum heterozygoten Alport-Syndrom (ehemals: thin basement membran nephropathie oder benigne familiäre Hämaturie). (uni-goettingen.de)
  • Mutations that affect the GBM's collagen α3α4α5(IV) components cause Alport syndrome (kidney disease with variable ear and eye defects) and its variants, including thin basement membrane nephropathy. (wustl.edu)
  • 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)
  • BACKGROUND: Pierson syndrome and X-linked Alport syndrome result from pathogenic variants in LAMB2 and COL4A5, respectively, and both affect basement membranes in the kidney and the eye. (bvsalud.org)
  • The resemblance of the ocular features in Pierson syndrome and X-linked Alport syndrome suggests that mutations in LAMB2 and COL4A5 have similar effects on basement membranes and the pathogenesis of ocular damage. (bvsalud.org)
  • 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)
  • Produced by a variety of cell types, extracellular vesicles (EVs) are able to transfer lipids, nucleic acids and proteins to recipient cells. (nature.com)
  • Cutis laxa (CL) is a connective tissue disorder characterised by excessive loose, wrinkled and inelastic skin due to structural defects in the elastic fibres or abnormal maturation and secretion of proteins involved [4] . (rbht.nhs.uk)
  • Using mouse models and intravital microscopy, we show that migrating PMNs expand and use the low expression regions (LERs) of matrix proteins in the vascular basement membrane (BM) for their transmigration. (tamu.edu)
  • Like most proteins, when collagen is heated, it loses all of its structure. (rcsb.org)
  • Laminin and type IV collagen are the two principal components of the basement membrane. (biogenex.com)
  • Laminin consists of a 220 kD subunit, which is disulfide-linked into larger complexes or even into the structural scaffolding of the basement membrane itself. (biogenex.com)
  • In surgical pathology, laminin can be used as a marker to demonstrate morphologic change of basement membrane, which is helpful for interpreting the invasion of malignant tumors. (biogenex.com)
  • The very different types of kidney diseases that result from mutations in collagen IV vs. laminin are likely due to very different pathogenic mechanisms. (wustl.edu)
  • Some carriers are unaffected, while others develop a less severe condition called thin basement membrane nephropathy, which is characterized by hematuria. (medlineplus.gov)
  • Collagen can help to reduce inflammation in the skin, which is a common cause of acne. (femina.in)
  • Collagen can help to improve the appearance of dark spots by promoting skin regeneration and reducing inflammation. (femina.in)
  • Pericytes regulate vascular basement membrane remodeling and govern neutrophil extravasation during inflammation. (tamu.edu)
  • During inflammation polymorphonuclear neutrophils (PMNs) traverse venular walls, composed of the endothelium, pericyte sheath and vascular basement membrane. (tamu.edu)
  • The clinical presentation varies according to the histologic type of inflammation, the size of the involved blood vessel segment, and the distribution of the involved vessels. (medscape.com)
  • 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)
  • This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. (wikipedia.org)
  • Collagen type IV molecules assemble to form a sheet-like network which is involved in maintaining the structural integrity of basement membranes. (uchicago.edu)
  • A variety of evidence, including ultrastructural immunolocalization, has previously shown that type VII collagen is a major structural component of anchoring fibrils, found immediately beneath the lamina densa of many epithelia. (elsevierpure.com)
  • We make many different kinds of collagen, which form long ropes and tough sheets that are used for structural support in mature animals and as pathways for cellular movement during development. (rcsb.org)
  • A different collagen--"type IV"--forms the structural basis of this membrane. (rcsb.org)
  • Procedure -Sections were immunolabeled with a monospecific antibody that cross-reacts with human and canine α6(IV) chains and examined by means of fluorescence microscopy. (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)
  • In the pursuit of finding biomarkers that predict treatment response, we evaluated the association between serum biomarkers of collagen and vimentin turnover and outcomes in metastatic melanoma patients treated with the anti-CTLA-4 antibody ipilimumab (IPI). (bmj.com)
  • This is a classic type II reaction in the Gell and Coombs classification of antigen-antibody reactions. (medscape.com)
  • This particular collagen IV subunit, however, is only found in a subset of basement membranes. (wikipedia.org)
  • In most patients, the autoantibody in Goodpasture syndrome is directed against a 28-kd monomeric subunit present within the noncollagenous domain of the alpha3 chain of type IV collagen (alpha3[IV]NC1). (medscape.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)
  • The basement membranes are complex structures that support layers of endothelium and epithelium. (medscape.com)
  • 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)
  • Our study showed that type IV collagen and, therefore the basement membrane, play fundamental roles in coordinating alveolar morphogenesis. (biomedcentral.com)
  • Resident cells within the lung such as fibroblasts and alveolar type II cells may be induced by cigarette smoke to synthesize chemokines attracting and potentially activating inflammatory cells. (atsjournals.org)
  • This type of collagen is the primary component of reticular fibers, which are found in your skin and blood vessels. (femina.in)
  • This extended network is capable of entrapping a large number of banded collagen fibers, microfibrils, and other stromal matrix components. (elsevierpure.com)
  • These "type I" collagen molecules associate side-by-side, like fibers in a rope, to form tough fibrils. (rcsb.org)
  • The dermatosparaxis type is caused by mutations in the ADAMTS2 gene. (rbht.nhs.uk)
  • Several studies have linked the consumption of collagen supplements to improved skin elasticity in women 35 years of age and older. (webmd.com)
  • Collagen plays a crucial role in maintaining skin elasticity, which is the ability of the skin to stretch and bounce back. (femina.in)
  • Supplementing with collagen can help improve skin elasticity and firmness (4) . (femina.in)
  • Results -Immunolabeling of the α6(IV) chain was not observed in GBM of 6 dogs ≤ 30 months old but was observed in GBM of the remaining 6 dogs, all of which were ≥ 45 months old. (avma.org)
  • Expression of the α6(IV) chain was not observed in GBM of the human subjects, regardless of the age of the subject. (avma.org)
  • Conclusions and Clinical Relevance -Results indicate that the α6(IV) chain is expressed in GBM of healthy dogs, but the expression is age-dependent. (avma.org)
  • This gene encodes the major type IV alpha collagen chain of basement membranes. (antibodies-online.com)
  • Abnormal α-chain structures destroy the integrity of the basement membrane, which initially leads to hematuria and later develops into moderate-to-severe proteinuria, progressive renal failure, high-pitched sensorineural hearing impairment, and eye disease. (researchsquare.com)
  • Hydroxyproline, which is critical for collagen stability, is created by modifying normal proline amino acids after the collagen chain is built. (rcsb.org)
  • however, only 70 can access electron microscopy, and 42 can conduct type IV collagen α5 chain staining. (bvsalud.org)
  • The building blocks are composed of three alpha subunits of collagen, which form a triple helix. (medscape.com)
  • Collagen is composed of three chains, wound together in a tight triple helix. (rcsb.org)
  • All contain a long stretch of triple helix connected to different types of ends. (rcsb.org)
  • A special amino acid sequence makes the tight collagen triple helix particularly stable. (rcsb.org)
  • Variants in these genes result in the production of abnormal versions of collagen IV in glomeruli. (medlineplus.gov)
  • 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)
  • Matrix metalloproteases (MMPs) are zinc-dependent proteinases that participate in extracellular matrix degradation 1-3 and favor the invasion and proliferation of tumor cells 4 . (bvsalud.org)
  • 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)
  • Kidney Transplantation Kidney transplantation is the most common type of solid organ transplantation. (msdmanuals.com)
  • Type IV collagen can be expressed as six different chains, alpha1 to alpha6. (medscape.com)
  • Type IV collagen consists of six different α-chains that interact to form three different heterotrimers (α1α1α2, α3α4α5, and α5α5α6) (Warady et al. (researchsquare.com)
  • A small portion of collagen, colored by atom (left) and colored to highlight the three chains (right). (rcsb.org)
  • This illustration depicts a basement membrane, which forms a tough surface that supports the skin and many organs. (rcsb.org)
  • 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)
  • In autosomal dominant CL, phenotypic variability is common with some patients carrying genetic variants in the ELN or FBLN5 genes [4] . (rbht.nhs.uk)
  • Autosomal recessive CL is the most common form of inherited neonatal CL and may be attributed to mutations in EFEMP2 ( FBLN4 ), FBLN5 and ATP6V0A2 genes [4] . (rbht.nhs.uk)
  • To date, adeno-associated virus (AAV) has been the most effective vector for retinal gene delivery because it elicits minimal immune response and can mediate long-term transgene expression in a variety of non-dividing retinal cell types. (molvis.org)
  • 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 vitreous, ILM, and retinal ECM all contain glycosaminoglycans (GAGs), while the vitreous and ILM contain collagens. (molvis.org)
  • Breast cancers are commonly divided into four molecular subtypes based on specific therapeutically actionable biomarkers and gene expression profiles 1 . (nature.com)
  • The expression of biomarkers can be detected intracellularly or on the cell membrane of melanoma cells or noncancer cells in association with the melanoma. (hindawi.com)
  • The vascular type results from mutations in the COL3A1 gene. (rbht.nhs.uk)
  • 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)
  • However, the poor sensitivity and specificity of those markers and many other molecules are serious limitations for their routine use in both early (AJCC stage I and II) and advanced stages of melanoma (AJCC stage III and IV). (hindawi.com)
  • The heads bind strongly together, head-to-head, and four collagen molecules associate together through their tails, forming an X-shaped complex. (rcsb.org)
  • 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)
  • A non-fibrillar collagen found in the structure of BASEMENT MEMBRANE. (uchicago.edu)
  • Abnormal versions of collagen IV in the eye can result in misshapen lenses and an abnormally colored retina. (medlineplus.gov)
  • Type IV collagen: structure, gene organization, and role in human diseases. (wikipedia.org)
  • While it may not be as well-known as the others, Type IV collagen plays an essential role in the health of your kidneys, inner ear, and eye lens. (femina.in)
  • Proximal tubule cells exposed to PMA for 2 h demonstrated a two- to threefold increase in Na/H antiporter mRNA (mRNANa/H) abundance 4 h later. (jci.org)
  • The Hudson Lab studies the structure and function of type IV Collagen, the major constituent of basement membranes. (vumc.org)
  • The gelatinases MMP-2 and MMP-9 are enzymes that will hydrolyse type IV collagen, the major component of basement membranes. (bmj.com)
  • This graph shows the total number of publications written about "Collagen Type IV" by people in this website by year, and whether "Collagen Type IV" was a major or minor topic of these publications. (uchicago.edu)
  • 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)
  • Collagen provides structure to our bodies, protecting and supporting the softer tissues and connecting them with the skeleton. (rcsb.org)
  • You can get collagen through foods as well as dietary supplements. (webmd.com)
  • Research is ongoing as to the specific health benefits of collagen supplements versus food collagen. (webmd.com)
  • Research indicates that taking collagen peptide supplements can increase bone mineral density in women who have gone through menopause . (webmd.com)
  • One study of 72 male participants showed that an exercise regimen combined with collagen supplements produced greater improvements than exercise alone. (webmd.com)
  • Experiment with different collagen supplements and topical products to find what works best for your skin combine collagen treatments with a healthy diet and lifestyle to get the most out of your skincare routine. (femina.in)
  • CONCLUSION: Our patient had a later onset form of Pierson syndrome or "FSGS type 5, with or without ocular abnormalities," consistent with his "milder" LAMB2 missense variant. (bvsalud.org)