826 results match your criteria Heavy Chain Disease Gamma

Unique aspects of adaptive immunity in camelids and their applications.

Mol Immunol 2021 Jun 19;134:102-108. Epub 2021 Mar 19.

Vaccine and Infectious Disease Organization and Department of Veterinary Microbiology, Western College of Veterinary Medicine, University of Saskatchewan, Saskatoon, Canada. Electronic address:

Members of the Camelidae have unique adaptive immunological features that are not widely observed in other species. All camelids are known to have three distinct IgG isotypes - IgG1, IgG2 and IgG3. While IgG1 has a conventional antibody structure, both IgG2 and IgG3 are devoid of light chains and instead possess hypervariable regions in their heavy chain (VHH), while lacking the typical CH1 domain found in heavy chains. Read More

View Article and Full-Text PDF

Gamma heavy chain disease associated with rheumatoid arthritis: a case report.

J Med Case Rep 2021 Mar 17;15(1):121. Epub 2021 Mar 17.

Internal Medicine Department, Hôtel-Dieu, Nantes University Hospital, University of Nantes, 1 place Alexis Ricordeau, 44093, Nantes, France.

Background: Gamma heavy chain disease (γ-HCD) is a monoclonal gammopathy defined by an abnormal clonal and isolated production of incomplete heavy chain gamma (γ), unable to bind with light chains kappa or lambda. This disease is rare and remains poorly described. Its association to lymphoid neoplasm is well established, but exceptional forms of γ-HCD may also accompany auto-immune diseases. Read More

View Article and Full-Text PDF

CREG1 improves the capacity of the skeletal muscle response to exercise endurance via modulation of mitophagy.

Autophagy 2021 Apr 18:1-17. Epub 2021 Apr 18.

Department of Cardiology and Cardiovascular Research Institute, General Hospital of Northern Theater Command, Shenyang, China.

CREG1 (cellular repressor of E1A-stimulated genes 1) is involved in tissue homeostasis and influences macroautophagy/autophagy to protect cardiovascular function. However, the physiological and pathological role of CREG1 in the skeletal muscle is not clear. Here, we established a skeletal muscle-specific knockout mouse model () by crossing the -floxed mice () with a transgenic line expressing recombinase under the muscle-specific (creatine kinase, muscle) promoter. Read More

View Article and Full-Text PDF

Follicular T-cell lymphoma mimicking lymphocyte-rich classic Hodgkin lymphoma: a case report of a diagnostic pitfall.

J Clin Exp Hematop 2021 Mar 15. Epub 2021 Mar 15.

Department of Pathology and Molecular Diagnostics, Aichi Cancer Center Hospital, Nagoya, Japan.

Follicular T-cell lymphoma (FTCL), one of the nodal T-cell lymphomas with T follicular helper (T) phenotype, is an uncommon disease. The diagnosis of FTCL is challenging on the distinction from the morphological mimics mostly exemplified by follicular lymphoma. Here, we described a case of FTCL that mimicked lymphocyte-rich classic Hodgkin lymphoma (LRCHL). Read More

View Article and Full-Text PDF

Loss of sarcomeric proteins via upregulation of JAK/STAT signaling underlies interferon-γ-induced contractile deficit in engineered human myocardium.

Acta Biomater 2021 05 8;126:144-153. Epub 2021 Mar 8.

Department of Biomedical Engineering, Duke University, 101 Science Drive, Durham, NC 27708, USA. Electronic address:

The level of circulating interferon-γ (IFNγ) is elevated in various clinical conditions including autoimmune and inflammatory diseases, sepsis, acute coronary syndrome, and viral infections. As these conditions are associated with high risk of myocardial dysfunction, we investigated the effects of IFNγ on 3D fibrin-based engineered human cardiac tissues ("cardiobundles"). Cardiobundles were fabricated from human pluripotent stem cell-derived cardiomyocytes, exposed to 0-20 ng/ml of IFNγ on culture days 7-14, and assessed for changes in tissue structure, viability, contractile force and calcium transient generation, action potential propagation, cytokine secretion, and expression of select genes and proteins. Read More

View Article and Full-Text PDF

Coming together at the hinges: Therapeutic prospects of IgG3.

MAbs 2021 Jan-Dec;13(1):1882028

Thayer School of Engineering, Dartmouth College , Hanover, NH, USA.

The human IgG3 subclass is conspicuously absent among the formats for approved monoclonal antibody therapies and Fc fusion protein biologics. Concern about the potential for rapid degradation, reduced plasma half-life, and increased immunogenicity due to marked variation in allotypes has apparently outweighed the potential advantages of IgG3, which include high affinity for activating Fc receptors, effective complement fixation, and a long hinge that appears better suited for low abundance targets. This review aims to highlight distinguishing features of IgG3 and to explore its functional role in the immune response. Read More

View Article and Full-Text PDF
February 2021

Effects of dietary ferulic acid supplementation on growth performance and skeletal muscle fiber type conversion in weaned piglets.

J Sci Food Agric 2021 Feb 14. Epub 2021 Feb 14.

Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Institute of Animal Nutrition, Sichuan Agricultural University, Chengdu, Sichuan, 611130, People's Republic of China.

Background: Ferulic acid (FA) is a common polyphenolic compound. The purpose of this study was to explore the effect of dietary FA supplementation on growth performance and muscle fiber type conversion in weaned piglets. In this study, eighteen 21-day-old DLY (Duroc × Landrace × Yorkshire) weaned piglets were randomly divided into control, 0. Read More

View Article and Full-Text PDF
February 2021

Exosome-Derived Mediators as Potential Biomarkers for Cardiovascular Diseases: A Network Approach.

Proteomes 2021 Feb 1;9(1). Epub 2021 Feb 1.

Department of Surgery and Physiology, Cardiovascular R&D Center, Faculty of Medicine of the University of Porto, Alameda Professor Hernâni Monteiro, 4200-319 Porto, Portugal.

Cardiovascular diseases (CVDs) are widely recognized as the leading cause of mortality worldwide. Despite the advances in clinical management over the past decades, the underlying pathological mechanisms remain largely unknown. Exosomes have drawn the attention of researchers for their relevance in intercellular communication under both physiological and pathological conditions. Read More

View Article and Full-Text PDF
February 2021

A New Mouse Model of Chronic Myocarditis Induced by Recombinant Bacille Calmette-Guèrin Expressing a T-Cell Epitope of Cardiac Myosin Heavy Chain-α.

Int J Mol Sci 2021 Jan 14;22(2). Epub 2021 Jan 14.

Tsukuba Primate Research Center, National Institutes of Biomedical Innovation, Health and Nutrition, Tsukuba 305-0843, Japan.

Dilated cardiomyopathy (DCM) is a potentially lethal disorder characterized by progressive impairment of cardiac function. Chronic myocarditis has long been hypothesized to be one of the causes of DCM. However, owing to the lack of suitable animal models of chronic myocarditis, its pathophysiology remains unclear. Read More

View Article and Full-Text PDF
January 2021

Light Chain Mutation Effects on the Dynamics of Thrombin.

J Chem Inf Model 2021 Feb 15;61(2):950-965. Epub 2021 Jan 15.

Department of Physics, Wake Forest University, Winston-Salem, North Carolina 27106 United States.

Thrombin plays an important role in the process of hemostasis and blood coagulation. Studies in thrombin can help us find ways to treat cancer because thrombin is able to reduce the characteristic hypercoagulability of cancer. Thrombin is composed of two chains, the light chain and the heavy chain. Read More

View Article and Full-Text PDF
February 2021

Gamma heavy chain disease complicated by pulmonary hypertension, which was successfully treated with lenalidomide.

BMJ Case Rep 2020 Nov 30;13(11). Epub 2020 Nov 30.

Hematology, Tazuke Kofukai, Medical Research Institute, Kitano Hospital, Osaka, Japan.

Heavy chain disease (HCD) is a rare B-cell proliferative neoplasm that is characterised by the production of truncated monoclonal immunoglobulin heavy chains without light chains. Gamma HCD is a subgroup of HCD. A 67-year-old man was admitted to our hospital with dyspnoea and lower leg oedema. Read More

View Article and Full-Text PDF
November 2020

[Gamma heavy chain disease associated with aggressive B lymphoma in the context of myelodysplastic syndrome].

Rev Med Interne 2020 Nov 20. Epub 2020 Nov 20.

Centre hospitalier Montauban, 84, avenue 11(e) Régiment Infanterie, 82000 Montauban, France.

Introduction: Heavy chain disease is a rare entity characterized by the production of incomplete immunoglobulin heavy chain without associated light chain. It is a B-cell lymphoproliferation, categorized according to the immunoglobulin involved. It is often associated with lymphomas but also with autoimmune diseases. Read More

View Article and Full-Text PDF
November 2020

Gamma-heavy chain disease.

Klin Onkol 2020 ;33(4):282-285

Background: Gamma-heavy chain disease is a rare disease, described so far in approximately 150 cases. The aim of this work was laboratory dia-gnostics of immunoglobulin heavy chain disease.

Materials And Methods: A 60-year-old patient was referred to the University Hospital in Ostrava for suspected marginal zone lymphoma from gastric bio-psy. Read More

View Article and Full-Text PDF
January 2020

Rapid Ex-Vivo Ciliogenesis and Dose-Dependent Effect of Notch Inhibition on Ciliogenesis of Respiratory Epithelia.

Biomolecules 2020 08 14;10(8). Epub 2020 Aug 14.

Department of Developmental Biology, University of Pittsburgh School of Medicine, 530 45th St, Pittsburgh, PA 15201, USA.

: Cilia are actin based cellular protrusions conserved from algae to complex multicellular organisms like Homo sapiens. Respiratory motile cilia line epithelial cells of the tracheobronchial tree, beat in a synchronous, metachronal wave, moving inhaled pollutants and pathogens cephalad. Their role in both congenital disorders like primary ciliary dyskinesia (PCD) to acquired disorders like chronic obstructive pulmonary disease (COPD) continues to evolve. Read More

View Article and Full-Text PDF

Grape seed proanthocyanidin extract promotes skeletal muscle fiber type transformation via AMPK signaling pathway.

J Nutr Biochem 2020 10 8;84:108462. Epub 2020 Jul 8.

Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Institute of Animal Nutrition, Sichuan Agricultural University, Chengdu, Sichuan 611130, P. R. China.

Transformation of skeletal muscle fiber type from fast twitch to slow twitch has significances for sustained contractile and stretchable events, energy homeostasis and antifatigue ability. However, the regulation of skeletal muscle fiber type transformation through nutritional intervention is still not fully spelled out. Grape seed proanthocyanidin extract (GSPE) has been widely reported to play a broader role in many aspects of diseases with its various pharmacological and health-promoting effects. Read More

View Article and Full-Text PDF
October 2020

Newcastle disease virus induces testicular damage and disrupts steroidogenesis in specific pathogen free roosters.

Vet Res 2020 Jun 29;51(1):84. Epub 2020 Jun 29.

Shanghai Veterinary Research Institute (SHVRI), Chinese Academy of Agricultural Sciences (CAAS), Shanghai, 200241, China.

Newcastle disease (ND), which is caused by Newcastle disease virus (NDV), can cause heavy economic losses to the poultry industry worldwide. It is characterised by extensive pathologies of the digestive, respiratory, and nervous systems and can cause severe damage to the reproductive system of egg-laying hens. However, it is unknown whether NDV replicates in the male reproductive system of chickens and induces any pathologies. Read More

View Article and Full-Text PDF

Plasma-Based Proteomics Profiling of Patients with Hyperthyroidism after Antithyroid Treatment.

Molecules 2020 Jun 19;25(12). Epub 2020 Jun 19.

Proteomics Resource Unit, Obesity Research Center, College of Medicine, King Saud University, P.O. Box 2925 (98), Riyadh 11461, Saudi Arabia.

Thyroid hormones critically modulate body homeostasis and haemostasis by regulating energy and metabolism. Previous studies have focused on individual pathways or proteins that are affected by increases in thyroid hormone levels, while an overall plasma proteomic signature of this increased level is lacking. Herein, an integrated untargeted proteomic approach with network analysis was used to identify changes in circulating proteins in the plasma proteome between hyperthyroid and euthyroid states. Read More

View Article and Full-Text PDF

Heavy Lifting: Nomenclature and Novel Therapy for Gamma Heavy Chain Disease and Other Heavy Chain Disorders.

Clin Lymphoma Myeloma Leuk 2020 08 6;20(8):493-498. Epub 2020 Mar 6.

Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH.

Heavy chain disorders are rare B-cell disorders and include heavy chain disease, heavy chain deposition disease, and heavy chain amyloidosis. These disorders share the pathognomonic finding of a truncated immunoglobulin heavy chain without an associated light chain in the serum or urine in the case of heavy chain disease or in the tissues in the case of heavy chain deposition disease and heavy chain amyloidosis but are clinically distinct entities. The clinical recognition and systematic approaches to these disorders are challenging because of the rarity of the diseases, lack of consensus on treatment approaches, and minimal data with novel therapy. Read More

View Article and Full-Text PDF

Performance of anti-CD19 chimeric antigen receptor T cells in genetically defined classes of chronic lymphocytic leukemia.

J Immunother Cancer 2020 03;8(1)

Central European Institute of Technology (CEITEC), Masaryk University, Brno, Czech Republic

Background: While achieving prolonged remissions in other B cell-derived malignancies, chimeric antigen receptor (CAR) T cells still underperform when injected into patients with chronic lymphocytic leukemia (CLL). We studied the influence of genetics on CLL response to anti-CD19 CAR T-cell therapy.

Methods: First, we studied 32 primary CLL samples composed of 26 immunoglobulin heavy-chain gene variable ()-unmutated (9 -mutated, 8 -mutated, and 9 without mutations in , , or ) and 6 -mutated samples without mutations in the above-mentioned genes. Read More

View Article and Full-Text PDF

Dual immunological and oxidative responses in Oreochromis niloticus fish exposed to lambda cyhalothrin and concurrently fed with Thyme powder (Thymus vulgaris L.): Stress and immune encoding gene expression.

Fish Shellfish Immunol 2020 May 9;100:208-218. Epub 2020 Mar 9.

Department of Fish Diseases and Management, Faculty of Veterinary Medicine, Zagazig University, Egypt.

The present study was performed to explore the immunotoxicological effects of the lambda cyhalothrin (LCH) insecticide and evaluate the efficiency of Thyme powder (TP) as a fish supplement in attenuation of LCH impact on Oreochromis niloticus (O. niloticus) fish. Fish was sampled following 30-days exposure to LCH (/ LC50: 0. Read More

View Article and Full-Text PDF

Antigen-Based Nano-Immunotherapy Controls Parasite Persistence, Inflammatory and Oxidative Stress, and Cardiac Fibrosis, the Hallmarks of Chronic Chagas Cardiomyopathy, in A Mouse Model of Infection.

Vaccines (Basel) 2020 Feb 21;8(1). Epub 2020 Feb 21.

Department of Microbiology and Immunology, The University of Texas Medical Branch (UTMB), Galveston, TX 77555-1070, USA.

Chagas cardiomyopathy is caused by (). We identified two candidate antigens (TcG2 and TcG4) that elicit antibodies and T cell responses in naturally infected diverse hosts. In this study, we cloned and in a nanovector and evaluated whether nano-immunotherapy (referred as nano2/4) offers resistance to chronic Chagas disease. Read More

View Article and Full-Text PDF
February 2020

Gamma Heavy Chain Disease - Diagnostic Challenges in an Unusual Case and a Brief Synopsis of the Current Literature.

Clin Lab 2020 Jan;66(1)

Background: Gamma heavy chain disease is a disorder characterized by the production of truncated heavy chains without associated light chains. Clinical manifestations differ greatly. Thus far no standard treatment has been formulated. Read More

View Article and Full-Text PDF
January 2020

Resveratrol regulates muscle fiber type conversion via miR-22-3p and AMPK/SIRT1/PGC-1α pathway.

J Nutr Biochem 2020 03 27;77:108297. Epub 2019 Nov 27.

Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Institute of Animal Nutrition, Sichuan Agricultural University, Chengdu, Sichuan 611130, PR China.

This study investigated the effects of resveratrol and miR-22-3p on muscle fiber type conversion in mouse C2C12 myotubes. Here we showed that resveratrol significantly increased the protein level of slow myosin heavy chain (MyHC) and the activities of succinic dehydrogenase and malate dehydrogenase, as well as markedly decreased the protein level of fast MyHC and the activity of lactate dehydrogenase. Immunofluorescence staining showed that resveratrol remarkably upregulated the number of slow MyHC-positive myotubes and downregulated the number of fast MyHC-positive myotubes, suggesting that resveratrol promoted muscle fiber type conversion from fast-twitch to slow-twitch in C2C12 myotubes. Read More

View Article and Full-Text PDF

Identification of gamma heavy chain disease using MALDI-TOF mass spectrometry.

Clin Biochem 2020 Mar 26;77:57-61. Epub 2019 Dec 26.

Department of Laboratory Medicine, Memorial Sloan Kettering Cancer Center, 327 E. 64th St., New York, NY 10065, USA.

We describe the use of MALDI-TOF mass spectrometry in the analysis of a suspected case of gamma heavy chain disease. The patient had an abnormal serum immunofixation result where a monoclonal gamma heavy chain band was present without a corresponding light chain. Analysis by MALDI-TOF mass spectrometry revealed large peaks in the spectrum following IgG-specific purification. Read More

View Article and Full-Text PDF

Gamma heavy chain disease (γ-HCD) as iatrogenic immunodeficiency- associated lymphoproliferative disorder: Possible emergent subtype of rheumatoid arthritis-associated γ-HCD.

J Clin Exp Hematop 2019 ;59(4):196-201

Gamma heavy chain disease (γ-HCD) is a rare B-cell neoplasm that produces a truncated immunoglobulin γ-heavy chain lacking the light chain. The clinical features of γ-HCD are heterogeneous, resembling different types of B-cell lymphomas. Although rheumatoid arthritis (RA) is one of the common underlying diseases of γ-HCD, the therapeutic modality for RA has changed greatly in recent years; therefore, γ-HCD as iatrogenic immunodeficiency-associated lymphoproliferative disorder (LPD) should be taken into consideration. Read More

View Article and Full-Text PDF

Heart non-specific effector CD4 T cells protect from postinflammatory fibrosis and cardiac dysfunction in experimental autoimmune myocarditis.

Basic Res Cardiol 2019 12 20;115(1). Epub 2019 Dec 20.

Department of Rheumatology, Center of Experimental Rheumatology, University Hospital Zurich, Zurich, Switzerland.

Heart-specific CD4 T cells have been implicated in development and progression of myocarditis in mice and in humans. Here, using mouse models of experimental autoimmune myocarditis (EAM) we investigated the role of heart non-specific CD4 T cells in the progression of the disease. Heart non-specific CD4 T cells were obtained from DO11. Read More

View Article and Full-Text PDF
December 2019

Arginine promotes porcine type I muscle fibres formation through improvement of mitochondrial biogenesis.

Br J Nutr 2020 03 29;123(5):499-507. Epub 2019 Nov 29.

Key Laboratory for Animal Disease-Resistance Nutrition of China Ministry of Education, Sichuan Agricultural University, Chengdu, Sichuan611130, People's Republic of China.

The present study aimed to investigate whether arginine (Arg) promotes porcine type I muscle fibres formation via improving mitochondrial biogenesis. In the in vivo study, a total of sixty Duroc × Landrace × Yorkshire weaning piglets with an average body weight of 6·55 (sd 0·36) kg were randomly divided into four treatments and fed with a basal diet or a basal diet supplemented with 0·5, 1·0 and 1·5 % l-Arg, respectively, in a 4-week trial. Results showed that dietary supplementation of 1·0 % Arg significantly enhanced the activity of succinate dehydrogenase, up-regulated the protein expression of myosin heavy chain I (MyHC I) and increased the mRNA levels of MyHC I, troponin I1, C1 and T1 (Tnni1, Tnnc1 and Tnnt1) in longissimus dorsi muscle compared with the control group. Read More

View Article and Full-Text PDF

A dendritic cell-targeted chimeric hepatitis B virus immunotherapeutic vaccine induces both cellular and humoral immune responses .

Hum Vaccin Immunother 2020 04 26;16(4):779-792. Epub 2019 Nov 26.

Vaccine and Infectious Disease Organization-International Vaccine Centre (VIDO-InterVac), University of Saskatchewan, Saskatoon, SK, Canada.

Chimigen® HBV Immunotherapeutic Vaccine (C-HBV), a recombinant chimeric fusion protein comprising hepatitis B virus (HBV) S1 and S2 surface antigen fragments, Core antigen and a murine monoclonal antibody heavy chain fragment (Fc), was designed and produced in Sf9 insect cells. C-HBV targets the host immune system through specific receptors present on dendritic cells (DCs) which facilitates antigen internalization, processing, and presentation on MHC class I and II to induce both cellular and humoral immune responses against HBV antigens. T cell responses, previously assessed by antigen presentation assays using human peripheral blood mononuclear cell (PBMC)-derived DCs and T cells from uninfected and HBV chronic-infected donors, demonstrated that C-HBV was highly immunogenic. Read More

View Article and Full-Text PDF

The quantification of antibody elements and receptors subunit expression using qPCR: The design of VH, VL, CH, CL, FcR subunits primers for a more holistic view of the immune system.

J Immunol Methods 2020 01 1;476:112683. Epub 2019 Nov 1.

Bioinformatics Institute, Agency for Science, Technology and Research (A*STAR), Singapore; p53 Laboratory, Agency for Science, Technology and Research (A*STAR), Singapore. Electronic address:

The expression levels of immunoglobulin elements and their receptors are important markers for health and disease. Within the immunoglobulin locus, the constant regions and the variable region families are associated with certain pathologies, yet a holistic view of the interaction between the expressions of the multiple genes remain to be fully characterized. There is thus an important need to quantify antibody elements, their receptors and the receptor subunits in blood (PBMC cDNA) for both screening and detailed studies of such associations. Read More

View Article and Full-Text PDF
January 2020