Glomerular collagen. Medical search. Frequent questions

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)
These people are said to have anti-glomerular basement membrane nephritis. (medlineplus.gov)
Goodpasture's name has been used in a more specific clinical condition known as Goodpasture disease, which is the pulmonary renal syndrome specifically associated with anti-glomerular basement membrane (anti-GBM) antibodies. (medscape.com)
The pathogenesis of anti-glomerular basement membrane (anti-GBM) disease (Goodpasture disease) is linked to the presence of autoantibodies that react with the alveolus in the lung and the basement membrane of the glomerulus in the kidney. (medscape.com)

Inflammatory processes (edema, fibrin deposition, capillary dilatation, migration of leukocytes and phagocytosis) and the later stages of wound healing (capillary proliferation, deposition of collagen, cicatrization) are inhibited. (drugs.com)
Chronic glomerulonephritis characterized histologically by proliferation of MESANGIAL CELLS , increase in the MESANGIAL EXTRACELLULAR MATRIX, and a thickening of the glomerular capillary walls. (lookformedical.com)
Glomerular capillary tuft + the epithelial cells → glomerulus. (letstalkmed.com)
JCI -What determines glomerular capillary permeability? (letstalkmed.com)
3. How does the glomerular capillary loop prevent the filtration of macromolecules? (abdominalkey.com)

Estimated glomerular filtration rate (eGFR), urinary sodium and serum AGT negatively correlated with urinary AGT.Multiple regression analysis indicated that low serum AGT, high urinary protein, urinary Ang II and urinary Col IV correlated significantly with high urinary AGT. (uwi.edu)
NSF involves the deposition of collagen in the skin and other organs in patients either on dialysis or with a glomerular filtration rate of less than 15 cc/min. (blogspot.com)
Clinical signs include persistent PROTEINURIA , from microalbuminuria progressing to ALBUMINURIA of greater than 300 mg/24 h, leading to reduced GLOMERULAR FILTRATION RATE and END-STAGE RENAL DISEASE. (lookformedical.com)
5. How is glomerular filtration rate (GFR) regulated in normal subjects on a day-to-day basis? (abdominalkey.com)

It is flanked by the podocytes and glomerular endothelial cells that both synthesize it and adhere to it. (wustl.edu)
Nephritic syndrome occurs due to inflammation and damage to endothelial cells of glomerular capillaries and can be triggered by several disease processes. (shiken.ai)

The thin membranous structure supporting the adjoining glomerular capillaries . (lookformedical.com)
Glomerular capillaries are interposed between an afferent and efferent arteriole. (abdominalkey.com)
Firstly, there "is obliteration of glomerular capillaries due to expansion of the mesangial region. (docksci.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 mechanism of structural changes of the kidney in human diabetic nephropathy (DN) and IgA nephropathy (IgAN) is not yet completely known, but excessive deposition of extracellular matrix (ECM), including various collagens, may be crucial to this process. (scienceopen.com)
It is composed of GLOMERULAR MESANGIAL CELLS and their EXTRACELLULAR MATRIX. (lookformedical.com)
Anti-Collagen Type IV has been prepared by immunoaffinity chromatography using immobilized antigens followed by extensive cross-adsorption against other collagens, human serum proteins and non-collagen extracellular matrix proteins to remove any unwanted specificities. (rockland.com)
Non-specific cross-reaction of anti-collagen antibodies with other human serum proteins or non-collagen extracellular matrix proteins is negligible. (rockland.com)
Several factors thought to be responsible for progression of glomerular disease affect mesangial cell turnover and extracellular matrix production and deposition. (docksci.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)

Inflammatory MCP-1 and fibrosis (collagen, fibronectin, plasminogen activator inhibitor-1, and osteopontin) renal biomarkers were significantly reduced in the CXA-10 (2.5 mpk) group. (aspetjournals.org)
Our results strongly support a pathologic role for HSP47 in both these diseases and that increased levels of HSP47 may play an important role in the excessive assembly of collagens resulting in glomerulosclerosis and interstitial fibrosis found in DN and IgAN patients. (scienceopen.com)
Glomerular scarring and interstitial fibrosis eventually result. (msdmanuals.com)

Collagen alpha-4(IV) chain is a protein that in humans is encoded by the COL4A4 gene. (wikipedia.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)
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)

Five renal biopsy specimens, diagnosed as minor glomerular abnormalities, were simultaneously studied as controls. (scienceopen.com)
Point mutations in the WT1 gene result in loss of its regulatory function, with the consequent abnormalities in glomerular formation and gonadal differentiation seen in Denys-Drash syndrome. (medscape.com)
Mutations that disrupt the second alternative splicing site of the WT1 gene alter the normal ratio of KTS-positive/negative isoforms from 2:1 to 1:2 and result in abnormalities in glomerular formation and gonadal differentiation seen in Frasier syndrome. (medscape.com)
Whether tubulointerstitial abnormalities found in primary glomerular diseases are a secondary event due to ischaemic tubular injury from disruption of the peritubular blood supply following glomerular destruction, or whether there is a destructive process that attacks both the tubulointerstitium and the glomerulus, remains to be proven. (docksci.com)

Involved in positive regulation of biosynthetic process and positive regulation of glomerular mesangial cell proliferation. (mcw.edu)

Extraglomerular mesangium lacis cells are located in the angle between the afferent and efferent glomerular arterioles. (lookformedical.com)

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)
Within the tubular lumen glomerular filtrate is modified by tubular cells. (abdominalkey.com)

these include loss of collagens alpha 4 and alpha 5(IV), retention of collagen alpha 1/2(IV), appearance of fibronectin and collagen VI, and increased levels of perlecan. (rupress.org)

Inflammation of the renal glomeruli ( KIDNEY GLOMERULUS ) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. (lookformedical.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)
Anti-GBM nephritis is a classic autoimmune disease characterized by the presence of circulating pathogenic autoantibodies directed against proteins in the glomerular and alveolar basement membranes. (medscape.com)
it is found only in a few specialized basement membranes, including the glomerular and alveolar basement membranes. (medscape.com)

The glycosaminoglycan (heparan sulfate) component of glomerular basement membranes from human kidneys of diabetic and nondiabetic subjects has been quantitated after isolation from protease digests of the membranes on OEAE-cellulose microcolumns. (diabetesjournals.org)
Since heparan sulfate has been implicated as a major component of the glomerular anionic filtration barrier, its decreased content in diabetic basement membranes may contribute to the proteinuria observed in this disease. (diabetesjournals.org)

Anti-GBM disease is an autoimmune disorder characterized by autoantibodies directed against the glomerular/alveolar basement membrane. (medscape.com)

Glomerular mesangium. (lookformedical.com)
A chronic form of glomerulonephritis characterized by deposits of predominantly IMMUNOGLOBULIN A in the mesangial area ( GLOMERULAR MESANGIUM ). (lookformedical.com)

increased deposition of collagens was often present in relation to a strong expression of HSP47, a stress protein known to regulate collagen synthesis/assembly. (scienceopen.com)
Synthesis and post-translational modifications of collagen, intermolecular crosslinks in collagen and elastin, proteoglycans. (muni.cz)

This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. (wikipedia.org)
Collagen IV is a major structural component of all basal laminae (BLs). (rupress.org)
Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. (cusabio.com)

Monoclonal antibodies specific for HSP47, type III collagen and type IV collagen were used to assess the relative expression of their proteins in paraffin-embedded renal sections by immunohistochemistry. (scienceopen.com)
Goodpasture syndrome is a severe disease of the lungs and the kidneys caused by antibodies to the alpha3(IV) collagen chains. (medlineplus.gov)
Antibodies are immune system proteins that normally attack foreign substances such as bacteria or viruses, but in people with Goodpasture syndrome, they target alpha3(IV) collagen chains. (medlineplus.gov)
It remains unclear why some people's immune systems make antibodies to their own collagen chains. (medlineplus.gov)
Over the years, tremendous gains have been made in our knowledge of the pathogenic mechanisms underlying anti-GBM nephritis, and the fact that the anti-GBM antibodies are directed against the noncollagenous globular domain (NC1 domain) of the alpha-3 chain of type IV GBM collagen is well known. (medscape.com)
[ 3 ] Again, the anti-GBM antibodies are directed against an epitope located at the NC1 domain at the C-terminal of the alpha-3 chain of type IV collagen. (medscape.com)
Antibodies reacting with the alpha-3 chain of type IV collagen can be detected in the serum and can be eluted from kidneys of patients with anti-GBM nephritis. (medscape.com)
Rockland produces highly active antibodies and conjugates to collagens. (rockland.com)
For these reasons, it is often extremely difficult to generate antibodies with specificities to collagens. (rockland.com)
Anti-GBM antibodies interact with the GBM glycoproteins, almost exclusively the epitope of the noncollagenous domain (NC1) of the α3 chain of type IV collagen. (medscape.com)

The COL4A3 gene provides instructions for making one piece of a protein called collagen IV. (medlineplus.gov)

Type IV collagen: structure, gene organization, and role in human diseases. (wikipedia.org)
Collagen IV α345 dysfunction in glomerular basement membrane diseases. (bvsalud.org)
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)
Nephrotic syndrome is typically caused by diseases which damage the filtration barrier at the glomerular basement membrane and is defined by a triad of proteinuria, hypoalbuminemia and oedema. (shiken.ai)
CKD secondary to glomerular disorders (CKD-GN), due to the known natural course of the diseases, is characterized by immune dysregulation, treatment with different immunosuppressive drugs, and a relapsing and remitting course [ 10 ]. (biomedcentral.com)

Our results indicate that reduced levels of proteins associated with foot processes and the glomerular slit diaphragm likely contribute, along with reduced levels of GBM collagens, to the nephropathy associated with NPS. (nih.gov)

Molecular and functional defects in kidneys of mice lacking collagen alpha 3(IV): implications for Alport syndrome. (rupress.org)
In the kidney, other types of collagen build up in the basement membranes, eventually scarring the kidneys and leading to kidney failure. (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)
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)
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)
The principal component of basement membrane is type IV collagen, which acts as a support structure and is composed of building blocks that are linked end-to-end. (medscape.com)
Type IV collagen can be expressed as six different chains, alpha1 to alpha6. (medscape.com)
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)
Hypertension, urinary protein, urinary Ang II and urinary Type IV collagen (Col IV) positively correlated with urinary AGT. (uwi.edu)
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)
Cause is a gene mutation affecting type IV collagen. (msdmanuals.com)
This antibody reacts with most mammalian Type IV collagens and has negligible cross-reactivity with Type I, II, III, V or VI collagens. (rockland.com)
Anti-Collagen Type IV has been tested by dot blot and IHC and is suitable for indirect trapping ELISA for quantitation of antigen in serum using a standard curve, immunoprecipitation, native (non-denaturing, non-dissociating) PAGE, immunohistochemistry, and western blotting for highly sensitive qualitative analysis. (rockland.com)

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)

This interaction results in complement activation with glomerular infiltration of polymorphonuclear leukocytes (PMNs) and monocytes. (medscape.com)

The syndrome was subsequently shown to be caused by an antibody response against antigens present in the glomerular basement membrane (GBM). (medscape.com)

We show here that mice lacking collagen alpha 3(IV) display a renal phenotype strikingly similar to Alport syndrome: decreased glomerular filtration (leading to uremia), compromised glomerular integrity (leading to proteinuria), structural changes in glomerular BL, and glomerulonephritis. (rupress.org)

Early investigations suggested that, after sufficient damage to total functional renal mass, a series of adaptive changes lead to progressive destruction of remaining nephrons, primarily through damage to glomeruli.l This "haemodynamic theory" states that compensatory glomerular hyperperfusion and hyperfiltration, together with glomerular hypertension, result in worsening proteinuria and progressive glomerular sclerosis. (docksci.com)

In humans, mutation of the collagen alpha 3, alpha 4, or alpha 5(IV) chain genes results in a delayed onset renal disease called Alport syndrome. (rupress.org)

By immunofluorescence, we investigated collagen IV chain composition of the glomerular basement membrane (GBM). (stanford.edu)

Specifically, this gene makes the alpha3(IV) chain of collagen IV. (medlineplus.gov)
Most of these variants change single protein building blocks (amino acids) in a region where the alpha3(IV) collagen chain combines with other collagen IV chains. (medlineplus.gov)
These changes in the structure or amount of alpha3(IV) chains severely impair the formation of alpha345(IV) collagen networks in the basement membranes of the kidney, inner ear, and eye. (medlineplus.gov)

The building blocks are composed of three alpha subunits of collagen, which form a triple helix. (medscape.com)
Because the antigenic epitope is hidden within the triple helix of the collagen, an environmental factor (eg, smoking, hydrocarbon exposure) presumably is required to unmask the cryptic Goodpasture antigen to the immune system. (medscape.com)

Arterial stiffness refers to a pathological process in which continuous and intermittent distention of the aorta with each heartbeat and during lifespan causes fatigue and fracture of the elastin fibers, and accumulation of collagen, leading to increased stiffening and decreased elasticity of the aorta's wall [ 1 ]. (biomedcentral.com)
In this study, we measured pulse wave velocity (PWV, m/sec), a validated marker of arterial stiffness, in CKD secondary to glomerular disorders (CKD-GN) compared with that of CKD secondary to hypertension or diabetes (CKD- HTN/DM). (biomedcentral.com)

In the glomerular BL of the kidney, the alpha 1 and alpha 2(IV) chains are replaced by the alpha 3-5(IV) chains as development proceeds. (rupress.org)
This chain combines with two other types of alpha (IV) chains (the alpha4 and alpha5 chains) to make alpha345(IV) collagen molecules. (medlineplus.gov)

Expression of glomerular basement membrane (GBM) collagens is reduced in Lmx1b(-/-) mice, suggesting one basis for NPS nephropathy. (nih.gov)

Acylated ghrelin treatment reversed TGF-& beta;1-induced myofibroblast-like phenotype, collagen contractile properties, and the upregulation of factors involved in HSC activation and fibrogenesis via PI3K/Akt/mTOR pathway. (unav.edu)

Heat shock protein (HSP) 47 has been identified as collagen-binding stress protein, shown to have a specific role in the intracellular processing of procollagen molecules during collagen assembly. (scienceopen.com)
Glomerular filtration barrier is a size and charge barrier (allowing only small and positively charged molecules to pass easily). (letstalkmed.com)

We suggest that these alterations contribute, along with loss of collagen IV isoforms per se, to renal pathology. (rupress.org)

Moreover, by inhibiting angiotensin II, a growth factor that is implicated in glomerular sclerosis, these drugs have a potential role in slowing sclerotic progression. (medscape.com)

Perivascular and glomerular immune complexes ( 2 , 4 , 5 ) can cause membranoproliferative glomerulonephritis ( 6 ) and segmental or circumferential arteritis ( 4 ) with mononuclear infiltration, fibrinoid necrosis and deposits, and increased intimal cellularity. (cdc.gov)

To determine whether increased deposition of collagens in human DN and IgAN is related to HSP47, we investigated the expression of HSP47 in renal biopsy and autopsy sections obtained from 22 DN and 45 IgAN patients. (scienceopen.com)
All patients with a glomerular disorder had a clinical-pathological diagnosis based on a recent kidney biopsy. (biomedcentral.com)

Some class-specific anti-collagens may be specific for three-dimensional epitopes which may result in diminished reactivity with denatured collagen or formalin-fixed, paraffin embedded tissues. (rockland.com)

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)

Methods Circulating (n = 179) and hepatic expression (n = 95) of ghrelin and LEAP-2 were measured in patients with severe obesity and available liver pathology analysis undergoing Roux-en-Y gastric bypass (RYGB). (unav.edu)

By double immunostaining, we found colocalization of collagens and their molecular chaperone HSP47 in the sclerotic glomeruli and tubulointerstitium in DN and IgAN. (scienceopen.com)

To assay for α3α4α5(IV) heterotrimer formation and secretion, we measured luminescence in cell lysates and culture supernatants from transfected cells.Immunostaining suggested that the collagen α3α4α5(IV) network was present throughout the patient's GBMs. (stanford.edu)

Rockland extensively purifies collagens for immunization from human and bovine placenta and cartilage by limited pepsin digestion and selective salt precipitation. (rockland.com)

Anatomy5

Diseases1

Chemicals and Drugs18

Anti-glomerular basement4

Capillary5

Filtration rate4

Endothelial cells2

Capillaries3

Human Glomerular Basement1

Extracellular matrix5

Laminin1

Fibrosis3

COL4A43

Abnormalities4

Mesangial cell1

Afferent and efferent1

Tubular2

Fibronectin1

Proliferation1

Alveolar basement membranes3

Heparan sulfate2

Autoantibodies directed1

Mesangium2

Synthesis2

Major structural component3

Antibodies10

COL4A31

Diseases5

Proteins1

Kidneys2

Type11

Mutations1

Complement activation1

Antibody1

Structural1

Proteinuria1

RENAL DISEASE1

Composition1

Alpha33

Triple helix2

Arterial Stiffness2

Chains2

Nephropathy1

Upregulation1

Molecules2

Isoforms1

Sclerosis1

Glomerulonephritis1

Biopsy2

Paraffin-embedded1

Genes1

Expression1

Molecular1

Secretion1

Cartilage1