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heavy chain deposition disease

Agnieszka Perkowska-Ptasinska, Dominika Deborska-Materkowska, Artur Bartczak, Tomasz Stompor, Tomasz Liberek, Barbara Bullo-Piontecka, Anna Wasinska, Agnieszka Serwacka, Marian Klinger, Jolanta Chyl, Michal Kuriga, Robert Malecki, Krzysztof Marczewski, Bogdan Hryniewicz, Tadeusz Gregorczyk, Monika Wieliczko, Stanislaw Niemczyk, Olga Rostkowska, Leszek Paczek, Magdalena Durlik
BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found...
November 25, 2016: BMC Nephrology
Taxiarchis V Kourelis, Surendra Dasari, Jason D Theis, Marina Ramirez-Alvarado, Paul J Kurtin, Morie A Gertz, Steven R Zeldenrust, Roman M Zenka, Ahmet Dogan, Angela Dispenzieri
The goal of this study was to investigate the frequency of use of light chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism. We evaluated clinic charts from 821 AL patients seen at the Mayo Clinic who had bone marrow, fat pad and solid organ tissue samples typed by liquid chromatography tandem mass spectrometry (LC-MS). We identified 701 patients with ALs and 120 with ALL. Overall, we were able to identify an IGVL gene in 87 (72%) patients with ALL and 573 (82%) patients with ALS...
November 16, 2016: Blood
Frank Bridoux, Vincent Javaugue, Sébastien Bender, Fannie Leroy, Pierre Aucouturier, Céline Debiais-Delpech, Jean-Michel Goujon, Nathalie Quellard, Amélie Bonaud, Marie Clavel, Patrick Trouillas, Florent Di Meo, Jean-Marc Gombert, Jean-Paul Fermand, Arnaud Jaccard, Michel Cogné, Guy Touchard, Christophe Sirac
Randall-type heavy chain deposition disease (HCDD) is a rare disorder characterized by tissue deposition of a truncated monoclonal immunoglobulin heavy chain lacking the first constant domain. Pathophysiological mechanisms are unclear and management remains to be defined. Here we retrospectively studied 15 patients with biopsy-proven HCDD of whom 14 presented with stage 3 or higher chronic kidney disease, with nephrotic syndrome in 9. Renal lesions were characterized by nodular glomerulosclerosis, with linear peritubular and glomerular deposits of γ-heavy chain in 12 patients or α-heavy chain in 3 patients, without concurrent light chain staining...
October 20, 2016: Kidney International
Yuhee Ryu, Li Jin, Hae Jin Kee, Zhe Hao Piao, Jae Yeong Cho, Gwi Ran Kim, Sin Young Choi, Ming Quan Lin, Myung Ho Jeong
Gallic acid, a type of phenolic acid, has been shown to have beneficial effects in inflammation, vascular calcification, and metabolic diseases. The present study was aimed at determining the effect and regulatory mechanism of gallic acid in cardiac hypertrophy and fibrosis. Cardiac hypertrophy was induced by isoproterenol (ISP) in mice and primary neonatal cardiomyocytes. Gallic acid pretreatment attenuated concentric cardiac hypertrophy. It downregulated the expression of atrial natriuretic peptide, brain natriuretic peptide, and beta-myosin heavy chain in vivo and in vitro...
October 5, 2016: Scientific Reports
Olga Balafa, Georgios Liapis, Paraskevi Pavlakou, Georgios Baltatzis, Rigas Kalaitzidis, Moses Elisaf
Kimmelstiel and Wilson originally described nodular glomerulosclerosis as the pathognomonic lesion of diabetic nephropathy. Nevertheless, nodular glomerulosclerosis pattern can rarely occur in non-diabetic patients. In such cases, the differential diagnosis includes membranoproliferative glomerulonephritis, light or heavy chain deposition disease, amyloidosis, fibrillary and immunotactoid glomerulonephritis and chronic hypoxic or ischemic conditions. In cases that the above entities cannot be proven, the term idiopathic nodular glomerulosclerosis is given...
September 22, 2016: Pathology, Research and Practice
Aaron C Petrey, Carol A de la Motte
Dynamic alterations of the extracellular matrix in response to injury directly modulate inflammation and consequently the promotion and resolution of disease. During inflammation, hyaluronan (HA) is increased at sites of inflammation where it may be covalently modified with the heavy chains (HC) of inter-α-trypsin inhibitor. Deposition of this unique, pathological form of HA (HC-HA) leads to the formation of cable-like structures that promote adhesion of leukocytes. Naive mononuclear leukocytes bind specifically to inflammation-associated HA matrices but do not adhere to HA constitutively expressed under homeostatic conditions...
November 18, 2016: Journal of Biological Chemistry
Xiulei Xue, Xiaobo Fan, Qingrong Qu, Guoqiu Wu
The variable regions of the camel heavy chain antibody, also known as nanobody is the smallest antibody with antigen-binding efficiency. CTGF is considered important during extracellular matrix deposition which was involved in the pathogenesis of fibrosis related diseases. There are several anti-CTGF-C nanobody drugs under developing in pharmacy. In this study, we described the screening of a novel anti-CTGF-C nanobody from the peripheral blood of immunized camel by phage display. The screened nanobody was further expressed and purified from E...
December 2016: AMB Express
Patricia A Shi, Erika Choi, Narendranath R Chintagari, Julia Nguyen, Xinhua Guo, Karina Yazdanbakhsh, Narla Mohandas, Abdu I Alayash, Elizabeth A Manci, John D Belcher, Gregory M Vercellotti
There is growing evidence that extracellular haemoglobin and haem mediate inflammatory and oxidative damage in sickle cell disease. Haptoglobin (Hp), the scavenger for free haemoglobin, is depleted in most patients with sickle cell disease due to chronic haemolysis. Although single infusions of Hp can ameliorate vaso-occlusion in mouse models of sickle cell disease, prior studies have not examined the therapeutic benefits of more chronic Hp dosing on sickle cell disease manifestations. In the present study, we explored the effect of Hp treatment over a 3-month period in sickle mice at two dosing regimens: the first at a moderate dose of 200 mg/kg thrice weekly and the second at a higher dose of 400 mg/kg thrice weekly...
November 2016: British Journal of Haematology
Rafaela Cabral Gonçalves Fabiano, Sérgio Veloso Brant Pinheiro, Ana Cristina Simões E Silva
BACKGROUND AND AIM: IgA nephropathy is one of the leading causes of primary glomerulonephritis worldwide and an important etiology of renal disease in young adults. IgA nephropathy is considered an immune complex-mediated disease. METHODS: This review article summarizes recent evidence on the pathophysiology of IgA nephropathy. RESULTS: Current studies indicate an ordered sequence of multi-hits as fundamental to disease occurrence. Altered glycan structures in the hinge region of the heavy chains of IgA1 molecules act as auto-antigens, potentially triggering the production of glycan-specific autoantibodies...
October 2016: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
Brittany Matuska, Suzy Comhair, Carol Farver, James Chmiel, Ronald J Midura, Tracey Bonfield, Mark E Lauer
Hyaluronan (HA) has been used in treatment of cystic fibrosis (CF) via a nebulizer and has demonstrated success in clinical outcomes. HA is an important glycosaminoglycan that is cross-linked by heavy chains (HCs) from inter-α-inhibitor during inflammation. HC cross-linked HA (HC-HA) becomes significantly more adhesive for leukocytes than non-cross-linked HA, which can enhance inflammation. Our studies tested the hypothesis that HC-HA is present in CF airways and that altered ratios of HC-HA to its degradation into relatively lower molecular weight HA contribute to the pathophysiology of chronic inflammation in CF...
October 2016: American Journal of Respiratory Cell and Molecular Biology
Barbora Knoppova, Colin Reily, Nicolas Maillard, Dana V Rizk, Zina Moldoveanu, Jiri Mestecky, Milan Raska, Matthew B Renfrow, Bruce A Julian, Jan Novak
IgA nephropathy (IgAN) is the most common primary glomerulonephritis, frequently leading to end-stage renal disease, as there is no disease-specific therapy. IgAN is diagnosed from pathological assessment of a renal biopsy specimen based on predominant or codominant IgA-containing immunodeposits, usually with complement C3 co-deposits and with variable presence of IgG and/or IgM. The IgA in these renal deposits is galactose-deficient IgA1, with less than a full complement of galactose residues on the O-glycans in the hinge region of the heavy chains...
2016: Frontiers in Immunology
Camille Cohen, Vincent Javaugue, Florent Joly, Bertrand Arnulf, Jean-Paul Fermand, Arnaud Jaccard, Christophe Sirac, Bertrand Knebelmann, Frank Bridoux, Guy Touchard
Monoclonal immunoglobulin (Ig) deposition disease (MIDD) is a rare complication of plasma cell disorders, defined by linear Congo red-negative deposits of monoclonal light chain (LCDD), heavy chain (HCDD) or both (LHCDD) along basement membranes. MIDD should be suspected in patients presenting with glomerular proteinuria and monoclonal gammopathy, but none of these criteria is necessary for the diagnosis although renal involvement is prominent. Since an abnormal serum κ/λ ratio is found in virtually all MIDD patients, including those with HCDD, serum free light chain assay should be included in the initial workup in patients older than 50 presenting with kidney disease...
June 2016: Néphrologie & Thérapeutique
Agnes B Fogo, Mark A Lusco, Behzad Najafian, Charles E Alpers
No abstract text is available yet for this article.
March 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Jessica Hemminger, Gyongyi Nadasdy, Anjali Satoskar, Sergey V Brodsky, Tibor Nadasdy
Immunofluorescence staining plays a vital role in nephropathology, but the panel of antibodies used has not changed for decades. Further classification of immunoglobulin (Ig)G-containing immune-type deposits with IgG subclass staining (IgG1, IgG2, IgG3, and IgG4) has been shown to be of diagnostic utility in glomerular diseases, but their value in the evaluation of renal biopsies has not been addressed systematically in large renal biopsy material. Between January 2007 and June 2014, using direct immunofluorescence, we stained every renal biopsy for the IgG subclasses if there was moderate to prominent glomerular IgG staining and/or IgG-predominant or IgG-codominant glomerular staining...
May 2016: American Journal of Surgical Pathology
Xian Chen, Yueliang Long, Xiaoping Zhu, Jun Li, Hong Liu, Shuguang Yuan, Letian Zhou
Monoclonal immunoglobulin deposition disease is rare in medical practice. The light and heavy chain deposition disease is characterized by deposition of monoclonal antibodies in the basement of membrane. Kidney is the most frequently involved organ. There was a male patient diagnosed as light and heavy chain deposition disease in department of Nephrology of the Second Xiangya Hospital, Central South University by renal biopsy. After treatment by oral prednisone, melphalan and thalidomide, the patient's proteinuria and serum creatinine decreased...
January 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Agnes B Fogo, Mark A Lusco, Behzad Najafian, Charles E Alpers
No abstract text is available yet for this article.
February 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Monica Centa, Sabrina Gruber, Daniel Nilsson, Konstantinos A Polyzos, Daniel K Johansson, Göran K Hansson, Daniel F J Ketelhuth, Christoph J Binder, Stephen Malin
OBJECTIVE: The V1 (VHS107.1.42) immunoglobulin heavy chain gene is thought to be critical in producing IgM natural antibodies of the T15-idiotype that protect against both atherosclerosis and infection from Streptococcus pneumoniae. Our aim was to determine whether genetic loss of the V1 gene increased atherosclerotic plaque burden in vivo because of a reduction in the T15-idiotype or other atheroprotective antibodies. APPROACH AND RESULTS: We crossed VHS107.1.42-deficient mice with the atherosclerosis-prone Apoe(-/-) and Ldlr(-/-) strains...
January 2016: Arteriosclerosis, Thrombosis, and Vascular Biology
Cardine N Nokwe, Manuel Hora, Martin Zacharias, Hisashi Yagi, Christine John, Bernd Reif, Yuji Goto, Johannes Buchner
The association of light chains (LCs) and heavy chains is the basis for functional antibodies that are essential for adaptive immune responses. However, in some cases, LCs and especially fragments consisting of the LC variable (VL) domain are pathologically deposited in fatal aggregation diseases. The two domains of the LC are connected by a highly conserved linker. We show here that, unexpectedly, the linker residue Arg108 affects the conformational stability and folding of both VLκ and LC constant (CLκ) domains...
November 6, 2015: Journal of Molecular Biology
Shun Manabe, Michiyasu Hatano, Masahide Yazaki, Kosaku Nitta, Michio Nagata
AH amyloidosis is a rare type of amyloidosis caused by deposition of monoclonal immunoglobulin heavy chain. The key diagnostic feature is positive immunostaining for a single class of immunoglobulin heavy chain. We report a case of AH amyloidosis with immunoglobulin G (IgG) λ monoclonal gammopathy that was diagnosed by liquid chromatography-tandem mass spectrometry (LC-MS/MS) after immunostaining of renal tissue for immunoglobulin heavy chain gave negative results. The molecular weight of the purified renal amyloid protein was ∼11kDa, which was determined by LC-MS/MS analysis to correspond to an amino acid sequence comprising the variable region and a truncated portion of the constant region of IgG heavy chain...
December 2015: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Leigh J Ellmers, Miriam T Rademaker, Christopher J Charles, Tim G Yandle, A Mark Richards
BACKGROUND: The (pro)renin receptor [(P)RR] is implicated in the pathogenesis of cardiovascular disease. We investigated the effects of (P)RR blockade after myocardial infarction (MI) in a mouse coronary-ligation model. METHODS AND RESULTS: Mice underwent sham control surgeries (n = 8) or induction of MI followed by 28 days' treatment with a vehicle control (n = 8) or (P)RR antagonist (n = 8). Compared with sham control subjects, MI + vehicle mice demonstrated reduced left ventricular (LV) ejection fraction (LVEF: P < ...
January 2016: Journal of Cardiac Failure
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