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https://www.readbyqxmd.com/read/29627782/pemphigoid-gestationis-successfully-treated-with-intravenous-immunoglobulin
#1
Filipa Tavares Almeida, Rita Sarabando, Joana Pardal, Celeste Brito
Pemphigoid gestationis (PG), also known as herpes gestationis , is a rare autoimmune blistering disease specific to pregnancy, which usually presents in the second or third trimesters and, in 15%-25% of cases, during the immediate postpartum period.1 Although the ethiopathogeny of PG is not fully clarified, most patients develop antibodies against a 180 kDa transmembrane hemidesmosomal protein (BP180; BPAG2; collagen XVII).2 PG has a strong association with human leucocyte antigens DR3 and DR4.3 We report a case of a 29-year-old female patient with PG successfully treated with intravenous immunoglobulin...
April 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29071428/ige-autoantibodies-and-their-association-with-the-disease-activity-and-phenotype-in-bullous-pemphigoid-a-systematic-review
#2
REVIEW
Ariadne Hadjikyriacou Saniklidou, Patrick J Tighe, Lucy C Fairclough, Ian Todd
Bullous pemphigoid (BP) is the most common autoimmune skin disease of blistering character. The underlying pathophysiological mechanism involves an immune attack, usually by IgG class autoantibodies, on the autoantigen BP 180/BPAg2, which is a type XVII collagen (COL17) protein acting as the adhesion molecule between the epidermis and the basement membrane of the dermis. About 40 years ago, following consistent findings of elevated total serum IgE levels in BP patients, it was hypothesized that IgE may be involved in the pathophysiology of BP...
January 2018: Archives of Dermatological Research
https://www.readbyqxmd.com/read/28590036/t-regulatory-cells-and-other-lymphocyte-subsets-in-patients-with-bullous-pemphigoid
#3
T Gambichler, A Tsitlakidon, M Skrygan, S Höxtermann, L Susok, S Hessam
BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease, and is associated with autoantibodies to the hemidesmosomal BP autoantigens BPAG1 and BPAG2. AIM: We aimed to investigate the significance of T regulatory cells and other lymphocyte subsets in patients with BP. METHODS: In total, 31 inpatients with BP were treated with systemic prednisolone in a tapered dose regimen, while 28 healthy individuals matched for age and sex served as the healthy control (HC) group...
August 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28353203/gene-structure-of-the-pregnancy-associated-glycoprotein-like-pag-l-in-the-eurasian-beaver-castor-fiber-l
#4
Aleksandra Lipka, Marta Majewska, Grzegorz Panasiewicz, Martyna Bieniek-Kobuszewska, Bozena Szafranska
The pregnancy-associated glycoprotein-like family (PAG-L) is a large group of chorionic products, expressed in the pre-placental trophoblast and later in the post-implantational chorionic epithelium, and are involved in proper placenta development and embryo-maternal interaction in eutherians. This study describes identification of the PAG-L family in the genome of the Eurasian beaver (Castor fiber L.), named CfPAG-L. We identified 7657 bp of the CfPAG-L gDNA sequence (Acc. No. KX377932), encompassing nine exons (1-9) and eight introns (A-H)...
September 2017: Functional & Integrative Genomics
https://www.readbyqxmd.com/read/28247089/bullous-pemphigoid-a-review-of-its-diagnosis-associations-and-treatment
#5
REVIEW
Philippe Bernard, Frank Antonicelli
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease in Western countries, and typically affects the elderly. BP is immunologically characterized by tissue-bound and circulating autoantibodies directed against either the BP antigen 180 (BP180, or BPAG2) or the BP antigen 230 (BP230, or BPAG1e), or even both, which are components of hemidesmosomes involved in the dermal-epidermal cohesion. Risk factors for BP include old age, neurologic diseases (dementia, Parkinson's disease, cerebrovascular disease), and some particular drugs, including loop diuretics, spironolactone and neuroleptics...
August 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/27891193/in-epithelial-cancers-aberrant-col17a1-promoter-methylation-predicts-its-misexpression-and-increased-invasion
#6
Pulari U Thangavelu, Tibor Krenács, Eloise Dray, Pascal H G Duijf
BACKGROUND: Metastasis is a leading cause of death among cancer patients. In the tumor microenvironment, altered levels of extracellular matrix proteins, such as collagens, can facilitate the first steps of cancer cell metastasis, including invasion into surrounding tissue and intravasation into the blood stream. However, the degree of misexpression of collagen genes in tumors remains understudied, even though this knowledge could greatly facilitate the development of cancer treatment options aimed at preventing metastasis...
2016: Clinical Epigenetics
https://www.readbyqxmd.com/read/26603373/bullous-pemphigoid-what-s-ahead
#7
REVIEW
Masutaka Furue, Takafumi Kadono
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease mainly affecting older individuals. Pathogenic autoantibodies preferentially target the non-collagenous 16A domain of collagen XVII (also called BP antigen 2, BPAG2) present in hemidesmosomes. The pathogenic anti-BPAG2 antibodies cause the dermal-epidermal separation in neonatal and adult mice as well as in cryosections of human skin. These experimental BP models stress a pivotal role for neutrophils and the Fcγ receptor of immunoglobulins...
March 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/26330528/the-molecular-architecture-of-hemidesmosomes-as-revealed-with-super-resolution-microscopy
#8
Leila Nahidiazar, Maaike Kreft, Bram van den Broek, Pablo Secades, Erik M M Manders, Arnoud Sonnenberg, Kees Jalink
Hemidesmosomes have been extensively studied with immunofluorescence microscopy, but owing to its limited resolution, the precise organization of hemidesmosomes remains poorly understood. We studied hemidesmosome organization in cultured keratinocytes with two- and three-color super-resolution microscopy. We observed that, in the cell periphery, nascent hemidesmosomes are associated with individual keratin filaments and that β4 integrin (also known as ITGB4) is distributed along, rather than under, keratin filaments...
October 15, 2015: Journal of Cell Science
https://www.readbyqxmd.com/read/26017636/molecular-architecture-and-function-of-the-hemidesmosome
#9
REVIEW
Gernot Walko, Maria J Castañón, Gerhard Wiche
Hemidesmosomes are multiprotein complexes that facilitate the stable adhesion of basal epithelial cells to the underlying basement membrane. The mechanical stability of hemidesmosomes relies on multiple interactions of a few protein components that form a membrane-embedded tightly-ordered complex. The core of this complex is provided by integrin α6β4 and P1a, an isoform of the cytoskeletal linker protein plectin that is specifically associated with hemidesmosomes. Integrin α6β4 binds to the extracellular matrix protein laminin-332, whereas P1a forms a bridge to the cytoplasmic keratin intermediate filament network...
June 2015: Cell and Tissue Research
https://www.readbyqxmd.com/read/25849406/combination-of-x-ray-crystallography-saxs-and-deer-to-obtain-the-structure-of-the-fniii-3-4-domains-of-integrin-%C3%AE-6%C3%AE-4
#10
Noelia Alonso-García, Inés García-Rubio, José A Manso, Rubén M Buey, Hector Urien, Arnoud Sonnenberg, Gunnar Jeschke, José M de Pereda
Integrin α6β4 is a major component of hemidesmosomes that mediate the stable anchorage of epithelial cells to the underlying basement membrane. Integrin α6β4 has also been implicated in cell proliferation and migration and in carcinoma progression. The third and fourth fibronectin type III domains (FnIII-3,4) of integrin β4 mediate binding to the hemidesmosomal proteins BPAG1e and BPAG2, and participate in signalling. Here, it is demonstrated that X-ray crystallography, small-angle X-ray scattering and double electron-electron resonance (DEER) complement each other to solve the structure of the FnIII-3,4 region...
April 2015: Acta Crystallographica. Section D, Biological Crystallography
https://www.readbyqxmd.com/read/25797172/ige-autoantibodies-in-bullous-pemphigoid-supporting-role-or-leading-player
#11
REVIEW
Hideyuki Ujiie
Bullous pemphigoid (BP) is a common autoimmune blistering skin disease in which two hemidesmosomal components--the transmembrane collagen XVII (BP180 or BPAG2) and the plakin family protein BP230 (BPAG1)--are targeted by autoimmunity. Of these, collagen XVII (COL17) is thought to be a major autoantigen, and vital roles of IgG autoantibodies in blister formation have been elucidated. However, BP shows distinct features, including pruritic urticarial erythema and eosinophilic infiltration, which may be independent of IgG-mediated autoimmunity...
April 2015: Journal of Dermatological Science
https://www.readbyqxmd.com/read/25487405/molecular-architecture-and-function-of-the-hemidesmosome
#12
REVIEW
Gernot Walko, Maria J Castañón, Gerhard Wiche
Hemidesmosomes are multiprotein complexes that facilitate the stable adhesion of basal epithelial cells to the underlying basement membrane. The mechanical stability of hemidesmosomes relies on multiple interactions of a few protein components that form a membrane-embedded tightly-ordered complex. The core of this complex is provided by integrin α6β4 and P1a, an isoform of the cytoskeletal linker protein plectin that is specifically associated with hemidesmosomes. Integrin α6β4 binds to the extracellular matrix protein laminin-332, whereas P1a forms a bridge to the cytoplasmic keratin intermediate filament network...
May 2015: Cell and Tissue Research
https://www.readbyqxmd.com/read/25178359/gestational-pemphigoid
#13
REVIEW
Laura Huilaja, Kaarin Mäkikallio, Kaisa Tasanen
Gestational pemphigoid (pemphigoid gestationis, PG) is a rare autoimmune skin disorder occurring characteristically during pregnancy. Autoantibodies against placental BP180 (also known as BPAG2 or collagen XVII) cause damage to the skin basement membrane, resulting in severe itching and blistering rash over the body and the extremities. The diagnosis of PG is confirmed by immunofluorescence analysis of a skin biopsy, while serum levels of pemphigoid antigen BP180 antibody can be used to assess disease activity...
September 2, 2014: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/24979241/a-retrospective-consecutive-case-series-study-on-the-effect-of-systemic-treatment-length-of-admission-time-and-co-morbidities-in-98-bullous-pemphigoid-patients-admitted-to-a-tertiary-centre
#14
Line Kibsgaard, Bjørn Bay, Mette Deleuran, Christian Vestergaard
Bullous pemphigoid (BP) is a common blistering disease caused by antibodies directed against hemi-desmosomal proteins BPAG1 and BPAG2. The disease is characterised by intense pruritus and blistering of the skin. The systemic treatment with the highest level of evidence for BP is systemic glucocorticoids. However, since the disease often occurs in the elderly patients, and since the most common co-morbidities are diabetes and neurological diseases, glucocorticoid-sparing drugs are often introduced. We retrospectively identified all BP patients admitted to our tertiary clinic over a 7-year period in order to register demography, treatment and co-morbidities...
March 2015: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/24434029/update-on-the-pathogenesis-of-bullous-pemphigoid-an-autoantibody-mediated-blistering-disease-targeting-collagen-xvii
#15
REVIEW
Wataru Nishie
Bullous pemphigoid (BP) is a common autoimmune blistering skin disorder that tends to affect the elderly. Autoantibodies (autoAbs) from BP patients react with two hemidesmosomal components: transmembrane collagen XVII (BP180 or BPAG2) and plakin family protein BP230 (BPAG1). Of these, collagen XVII (COL17) is thought to be a major autoantigen. The binding of autoAbs to COL17 following the activation of complements and inflammatory pathways eventually leads to the degradation of COL17, and this has been regarded as the main pathogenesis of BP...
March 2014: Journal of Dermatological Science
https://www.readbyqxmd.com/read/24278048/suspicion-of-pulmonary-embolism-during-treatment-of-pemphigoid-gestationis
#16
Magdalena Podolec-Rubiś, Marta Wołek, Paweł Brzewski, Anna Wojas-Pelc
Pemphigoid gestationis (PG), first described in 1872 by Laws Milton, is a rare autoimmune bullous disease of pregnancy and puerperium. Pemphigoid gestationis has been estimated to occur in 1: 50 000 pregnancies and usually presents in the second or third trimester with exacerbation after partum. This disease is caused by circulating IgG1 immunoglobulin against hemidesmosomal protein bullous pemphigoid BP180 (BPAG2) type XVII collagen and less frequently BP230. We present a case of pemphigoid gestationis in primigravida with complications during treatment due to the suspicion of pulmonary embolism...
February 2013: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/23337823/bullous-pemphigoid-igg-induces-bp180-internalization-via-a-macropinocytic-pathway
#17
Sho Hiroyasu, Toshiyuki Ozawa, Hiromi Kobayashi, Masamitsu Ishii, Yumi Aoyama, Yasuo Kitajima, Takashi Hashimoto, Jonathan C R Jones, Daisuke Tsuruta
Bullous pemphigoid (BP) is an autoimmune blistering skin disease induced by pathogenic autoantibodies against a type II transmembrane protein (BP180, collagen type XVII, or BPAG2). In animal models, BP180 autoantibody-antigen interaction appears insufficient to develop blisters, but involvement of complement and neutrophils is required. However, cultured keratinocytes treated with BP-IgG exhibit a reduction in the adhesive strength and a loss of expression of BP180, suggesting that the autoantibodies directly affect epidermal cell-extracellular matrix integrity...
March 2013: American Journal of Pathology
https://www.readbyqxmd.com/read/22718344/p2y2-receptor-inhibits-egf-induced-mapk-pathway-to-stabilise-keratinocyte-hemidesmosomes
#18
Emilie Faure, Françoise Garrouste, Fabrice Parat, Sylvie Monferran, Ludovic Leloup, Gilbert Pommier, Hervé Kovacic, Maxime Lehmann
α6β4 integrin is the main component of hemidesmosomes (HD) that stably anchor the epithelium to the underlying basement membrane. Epithelial cell migration requires HD remodelling, which can be promoted by epidermal growth factor (EGF). We previously showed that extracellular nucleotides inhibit growth factor-induced keratinocyte migration. Here, we investigate the effect of extracellular nucleotides on α6β4 integrin localisation in HD during EGF-induced cell migration. Using a combination of pharmacological inhibition and gene silencing approaches, we found that UTP activates the P2Y2 purinergic receptor and Gαq protein to inhibit EGF/ERK1/2-induced cell migration in keratinocytes...
September 15, 2012: Journal of Cell Science
https://www.readbyqxmd.com/read/22377765/transcutaneous-gene-gun-delivery-of-hnc16a-induces-bpag2-specific-tolerance
#19
Monika Ettinger, Doris Peckl-Schmid, Christina Gruber, Martin Laimer, Josef Thalhamer, Helmut Hintner, Iris K Gratz, Johann W Bauer
Immune recognition and rejection of tissues expressing transfected genes is a major complication of gene replacement therapy for inherited genetic disorders. Owing to the high immunogenicity of human bullous pemphigoid antigen 2 (hBPAG2), the induction and maintenance of tolerance to this neo-antigen is essential to deliver the gene product to patients with epidermolysis bullosa junctionalis. In a skin grafting mouse model, we used gene gun transfection with a construct encoding hNC16A, the immunodominant domain of hBPAG2, to induce antigen-specific immune tolerance...
June 2012: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/22137222/bullous-pemphigoid-from-the-clinic-to-the-bench
#20
REVIEW
Giovanni Di Zenzo, Rocco Della Torre, Giovanna Zambruno, Luca Borradori
Bullous pemphigoid (BP) constitutes the most frequent autoimmune subepidermal blistering disease. It is associated with autoantibodies directed against the BP antigens 180 (BP180, BPAG2) and BP230 (BPAG1-e). The pathogenicity of anti-BP180 antibodies has been convincingly demonstrated in animal models. The clinical features of BP are extremely polymorphous. The diagnosis of BP critically relies on immunopathologic findings. The recent development of novel enzyme-linked immunosorbent assays has allowed the detection of circulating autoantibodies with relatively high sensitivity and specificity...
January 2012: Clinics in Dermatology
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