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https://www.readbyqxmd.com/read/29310777/basement-membrane-collagen-iv-isolation-of-functional-domains
#1
Sergei P Boudko, Neonila Danylevych, Billy G Hudson, Vadim K Pedchenko
Collagen IV is a major constituent of basement membranes, specialized form of extracellular matrix that provides a mechanical support for tissues, serves as a polyvalent ligand for cell adhesion receptors and as a scaffold for other proteins, and plays a key role in tissue genesis, differentiation, homeostasis, and remodeling. Collagen IV underlies the pathogenesis of several human disorders including Goodpasture's disease, Alport's syndrome, diabetic nephropathy, angiopathy, and porencephaly. While the isolation of the collagen IV molecules from tissues is an ultimate prerequisite for structural and functional studies, it has been always hampered by the protein insolubility due to extensive intermolecular crosslinking and noncovalent associations with other components of basement membranes...
2018: Methods in Cell Biology
https://www.readbyqxmd.com/read/29290619/-a-giant-cell-arteritis-revealing-a-goodpasture-s-syndrome
#2
Pascaline M Alix, Amélie Belloi, Émilie Berthoux, Frédérique Dijoud, Emmanuel Villar, Caroline Pariset
Goodpasture's syndrome is a triad of anti-glomerular basement membrane (anti-GBM) circulating antibodies, glomerulonephritis and pulmonary hemorrhage. We reported a 65-year-old woman with headaches, asthenia and weight loss. Giant cell arteritis was confirmed by temporal artery biopsy. The patient had associated renal condition with moderate acute renal failure, proteinuria and haematuria. Renal biopsy showed extracapillary glomerulonephritis and linear staining of immunoglobulins G along glomerular basement membrane...
December 28, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29244791/management-and-outcomes-of-childhood-goodpasture-s-disease
#3
Carmela P Menzi, Barbara S Bucher, Mario G Bianchetti, Gianluigi Ardissino, Giacomo D Simonetti
BACKGROUND: In an attempt to improve knowledge about childhood Goodpasture's disease, we performed a retrospective analysis of patients with Goodpasture's disease from several pediatric nephrology centers. METHODS: We analyzed the responses to 27 questions that elicited information about the following: incidence, demographics, patient history and clinical presentation, diagnostics performed, acute and chronic therapy, course of disease and outcome. RESULTS: Goodpasture's disease, which is extremely rare in this age group, may manifest in 2-year-old toddlers and does not typically present with pulmonary findings before puberty...
December 15, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29124323/-goodpasture-syndrome-ocular-manifestation-in-a-young-man
#4
C Lommatzsch, A Lommatzsch, C Heinz, A Heiligenhaus
Goodpasture syndrome (GS) is a rare organ-specific autoimmune disease that may consist of glomerulonephritis and pulmonary hemorrhage. Only few case reports have been published on ocular symptoms in the context of GS. This is a case report of a 22-year-old male with GS who was referred to our practice because of visual disturbances. The anamnesis revealed pulmonary hemorrhage, acute renal failure and nephrotic syndrome, which was treated by immunosuppressive therapy with additional plasmapheresis. Ophthalmoscopically, bilateral cotton wool spots were found along the vessels and bilateral retinal hemorrhages predominantly at the posterior pole...
November 9, 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/29104431/a-case-of-rare-diffuse-alveolar-hemorrhage-and-review-of-literature
#5
Viviana Scollo, Luca Zanoli, Elisa Russo, Giulio Distefano, Francesco Rapisarda
It is well known that some disorders can cause concomitant kidney dysfunction with lung involvement. These syndromes, characterized by the simultaneous presence of intra-alveolar hemorrhage and acute glomerulonephritis, are caused by numerous and variable disorders. The most frequent are the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and Goodpasture syndrome. A quick discerning of the underlying causes and initiation of adequate treatment is crucial to prevent acute respiratory failure and irreversible loss of renal function...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29034872/particularities-of-goodpasture-syndrome
#6
Friedrich Lübbecke
No abstract text is available yet for this article.
September 29, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28927772/-antiglomerular-basement-disease-in-children-literature-review-and-therapeutic-options
#7
G Dorval, S Guérin, L Berteloot, S Krid, R Salomon, L Galmiche-Rolland, O Boyer
Antiglomerular basement membrane glomerulonephritis is a rare autoimmune disease characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage (Goodpasture syndrome). The disease is caused by autoantibodies (classically IgGs) directed against the α3 subunit of type IV collagen. This is a rare disease in the adult population and extremely rare in children, with a reported cumulative annual incidence at 1/10(6) people/year. Among scarce reported pediatric cases (n=31), most are girls (M/F sex ratio, 1:4), and the mean age at diagnoses is 9...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28886586/a-murine-ig-light-chain-transgene-reveals-igkv3-gene-contributions-to-anti-collagen-types-iv-and-ii-specificities
#8
Amy G Clark, Inge M Worni-Schudel, Francesca M Korte, Mary H Foster
A subset of autoimmune diseases result from autoantibodies targeting epitopes on matrix collagen. The most extensively studied are anti-glomerular basement membrane glomerulonephritis (or its systemic counterpart Goodpasture's disease) that destroys kidneys and lungs, and rheumatoid arthritis that leads to disabling arthritis. Autoantibodies in these disorders bind evolutionarily conserved conformational epitopes on the noncollagenous domain 1 (NC1) of the alpha3 chain of type IV [alpha3(IV)NC1] collagen in glomerular and alveolar basement membranes, and on native or citrullinated type II collagen (CII) in joint cartilage, respectively...
November 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28863794/immunoadsorption-in-autoimmune-diseases-affecting-the-kidney
#9
REVIEW
Georg Stummvoll, Martin Aringer, Ammon Handisurya, Kurt Derfler
Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Although past data have shown efficacy and biocompatibility of immunoadsorption in (renal) SLE, it is still an experimental and expensive procedure, and evidence from randomized controlled trials is needed...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28853702/familial-antiglomerular-basement-membrane-disease-in-zero-human-leukocyte-antigen-mismatch-siblings%C3%A2
#10
Andrea Angioi, Wisit Cheungpasitporn, Sanjeev Sethi, An S De Vriese, Nicola Lepori, Thomas R Schwab, Fernando C Fervenza
Reported cases of familial Antiglomerular basement membrane (anti-GBM) disease are extremely rare. The single gene mutations that may play a role in the development of familial anti-GBM disease are currently unidentified. While human leukocyte antigen (HLA)-DR15 is known to be associated with an increased risk of anti-GBM disease, HLA types in patients with familial anti-GBM disease have never been reported. We present a case of a 65-year-old woman with rapidly-progressive glomerulonephritis and pulmonary involvement, consistent with Goodpasture's syndrome...
November 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28796467/anti-glomerular-basement-membrane-antibody-diagnostics-in-a-large-cohort-tertiary-center-should-we-trust-serological-findings
#11
Abdulla Watad, Nicola Luigi Bragazzi, Kassem Sharif, Ora Shovman, Boris Gilburd, Howard Amital, Yehuda Shoenfeld
BACKGROUND: Anti-glomerular basement membrane (GBM) antibody disease, or Goodpasture's disease, is the clinical manifestation of the production of anti-GBM antibodies, which causes rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Anti-GBM antibody detection is mandatory for the diagnosis of Goodpasture's disease either from the serum or kidney biopsy. Renal biopsy is necessary for disease confirmation; however, in cases in which renal biopsy is not possible or is delayed, serum detection of anti-GBM antibody is the only way for diagnosis...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28765179/status-epilepticus-as-the-initial-presentation-of-antibody-negative-goodpasture-s-syndrome
#12
Ingrid Pl Ting, Sanihah Abdul Halim, Azreen Adnan, Hasnan Jaafar
Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28602810/apheresis-to-treat-systemic-vasculitis
#13
Julie Moussi-Frances, Marion Sallée, Noémie Jourde-Chiche
Apheresis has been used in the treatment of severe systemic vasculitides, in conjunction with immunosuppressive therapies, for over 40 years. The aim is to rapidly remove autoantibodies or circulating immune complexes from the plasma. The two main indications at present are vasculitis associated with Antineutrophil Cytoplasmic Antibodies (ANCAs) manifesting as severe renal involvement and/or intraalveolar hemorrhage and antiglomerular basement membrane disease (Goodpasture syndrome). The ongoing PEXIVAS randomized controlled trial is assessing plasmapheresis to treat ANCA-associated vasculitis with or without severe renal involvement or intraalveolar hemorrhage...
June 7, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28573056/antiglomerular-basement-membrane-disease-in-a-pediatric-patient-a-case-report-and-review-of-the-literature
#14
Vimal Master Sankar Raj, Diana Warnecke, Julia Roberts, Sarah Elhadi
Goodpasture's syndrome (GPS) remains a very rare disease entity in the pediatric population characterized by the presence of pulmonary hemorrhage and rapidly evolving glomerulonephritis. We hereby describe the case of a 2-year-old girl who presented with renal failure and was diagnosed with GPS. A brief review of the literature in regard to data on demographics, pathogenesis, clinical features, diagnosis, treatment, and prognosis for renal recovery is also provided.
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28570922/the-critical-amino-acids-of-a-nephritogenic-epitope-on-human-goodpasture-autoantigen-for-binding-to-hla-drb1-1501
#15
Qiu-Hua Gu, Xiao-Yu Jia, Jian-Nan Li, Fang-Jin Chen, Zhao Cui, Ming-Hui Zhao
BACKGROUND: Anti-GBM disease is caused by autoimmunity to Goodpasture antigen on α3(IV)NC1 and had strong associations with HLA-DRB1*1501. Previous studies identified α3127-148 (P14: TDIPPCPHGWISLWKGFSFIMF) as a T cell epitope. The present study was aimed to investigate the binding capacity of P14 to HLA-DRB1*1501 and the critical amino acids for this binding. METHODS: A line of EBV-transformed human B cells homozygous for HLA-DRB1*1501 was used to detect the binding capacity of peptides to HLA-DRB1*1501 using flow cytometry analysis...
August 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28550388/antibodies-against-linear-epitopes-on-goodpasture-autoantigen-in-patients-with-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#16
Xiao-Yu Jia, Jun-Tao Yu, Shui-Yi Hu, Jian-Nan Li, Miao Wang, Chen Wang, Min Chen, Zhao Cui, Ming-Hui Zhao
In a substantial number of patients with crescentic glomerulonephritis, both anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) are detected simultaneously. ANCA is presumed to be the initial event but the mechanism is unknown. In the present study, we investigated the antibodies against linear epitopes on Goodpasture autoantigen in sera from patients with ANCA-associated vasculitis, aiming to reveal the mechanisms of the coexistence of the two kinds of autoantibodies...
May 26, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28515462/autoimmunity-hla-mediated-protection-in-goodpasture-disease
#17
Ellen F Carney
No abstract text is available yet for this article.
July 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28502986/autoimmunity-hla-mediated-protection-in-goodpasture-disease
#18
Ellen F Carney
No abstract text is available yet for this article.
July 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28467828/dominant-protection-from-hla-linked-autoimmunity-by-antigen-specific-regulatory-t-cells
#19
Joshua D Ooi, Jan Petersen, Yu H Tan, Megan Huynh, Zoe J Willett, Sri H Ramarathinam, Peter J Eggenhuizen, Khai L Loh, Katherine A Watson, Poh Y Gan, Maliha A Alikhan, Nadine L Dudek, Andreas Handel, Billy G Hudson, Lars Fugger, David A Power, Stephen G Holt, P Toby Coates, Jon W Gregersen, Anthony W Purcell, Stephen R Holdsworth, Nicole L La Gruta, Hugh H Reid, Jamie Rossjohn, A Richard Kitching
Susceptibility and protection against human autoimmune diseases, including type I diabetes, multiple sclerosis, and Goodpasture disease, is associated with particular human leukocyte antigen (HLA) alleles. However, the mechanisms underpinning such HLA-mediated effects on self-tolerance remain unclear. Here we investigate the molecular mechanism of Goodpasture disease, an HLA-linked autoimmune renal disorder characterized by an immunodominant CD4+ T-cell self-epitope derived from the α3 chain of type IV collagen (α3135-145)...
May 11, 2017: Nature
https://www.readbyqxmd.com/read/28459999/diagnostic-and-management-challenges-in-goodpasture-s-anti-glomerular-basement-membrane-disease
#20
Scott R Henderson, Alan D Salama
Goodpasture's or anti-glomerular basement membrane (GBM) disease is classically characterized by the presence of circulating autoantibodies directed against the non-collagenous domain of the α3 chain of type IV collagen, targeting glomerular and alveolar basement membranes, and associated with rapidly progressive crescentic glomerulonephritis, with alveolar haemorrhage in over half the patients. However, there are increasing examples of variants or atypical presentations of this disease, and novel therapeutic options have been proposed, which nephrologists should be aware of...
April 28, 2017: Nephrology, Dialysis, Transplantation
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