Results 71 to 80 of about 36,985 (244)

Why has it been so difficult to prove the efficacy of alpha-1-antitrypsin replacement therapy? Insights from the study of disease pathogenesis

Drug Design, Development and Therapy, 2011
Jennifer A Dickens, David A LomasDepartment of Medicine, University of Cambridge, Cambridge Institute for Medical Research, Wellcome Trust/MRC Building, Cambridge, UKAbstract: Alpha-1-antitrypsin is the most abundant circulating protease inhibitor. It is
Dickens JA, Lomas DA
doaj  

Protective role of the alpha-1-antitrypsin in intervertebral disc degeneration

Journal of Orthopaedic Surgery and Research, 2021
Background Intervertebral disc degeneration is a complex disease with high prevalence. It suggests that cell death, senescence, and extracellular matrix degradation are involved in the pathogenesis. Alpha-1 antitrypsin (AAT), a serine protease inhibitor,
Weikun Liu, Yanfu Wang
doaj   +1 more source

An antibody raised against a pathogenic serpin variant induces mutant-like behaviour in the wild-type protein. [PDF]

, 2015
A monoclonal antibody (mAb) that binds to a transient intermediate may act as a catalyst for the corresponding reaction; here we show this principle can extend on a macro-molecular scale to the induction of mutant-like oligomerisation in a wild-type ...
Faull, SV, Haq, I, Irving, JA, Kotov, VR, Lomas, DA, Miranda, E, Motamedi-Shad, N, Perez, J   +7 more
core   +1 more source

Mass Spectrometry Structural Proteomics Enabled by Limited Proteolysis and Cross‐Linking

Mass Spectrometry Reviews, EarlyView.
ABSTRACT The exploration of protein structure and function stands at the forefront of life science and represents an ever‐expanding focus in the development of proteomics. As mass spectrometry (MS) offers readout of protein conformational changes at both the protein and peptide levels, MS‐based structural proteomics is making significant strides in the
Haiyan Lu, Zexin Zhu, Lauren Fields, Hua Zhang, Lingjun Li   +4 more
wiley   +1 more source

Diagnosis of alpha-1-antitrypsin deficiency by DNA analysis of children with liver disease

Arquivos de Gastroenterologia, 2001
Background - Alpha-1-antitrypsin deficiency is a genetic disorder which is transmitted in a co-dominant, autosomal form. Alpha-1-antitrypsin deficiency affects mainly the lungs and the liver leading, in the latter case, to neonatal cholestasis, chronic ...
De TOMMASO Adriana Maria Alves, ROSSI Cláudio Lúcio, ESCANHOELA Cecília Amélia Fazzio, SERRA Heliane Guerra, BERTUZZO Carmen Sílvia, HESSEL Gabriel   +5 more
doaj  

Sweet syndrome with pulmonary involvement in a patient with myelodysplastic syndrome [PDF]

, 2020
We report a patient with Sweet syndrome involving the pulmonary system in the context of myelodysplastic syndrome. Although Sweet syndrome may involve a variety of organ systems, the pulmonary system is rarely affected and can result in poor clinical ...
Bordelon, Jenna, Ho, Jonhan, James, Alaina, Karunamurthy, Arivarasan, Kazlouskaya, Viktoryia, Khosravi, Hasan, Mizes, Alicia, Patton, Timothy   +7 more
core  

The Plasma Free Fatty Acid Carrier Proteome is Altered in Diet‐Induced and Genetically Obese Mice

Obesity, EarlyView.
ABSTRACT Objective We aimed to examine the effect of obesity on the plasma free fatty acid (FFA) carrier proteome in mice. Methods From 6 to 20 weeks of age, male and female C57BL/6J wild‐type mice were fed either a low‐fat diet (10% of kilocalories from fat) or a high‐fat diet (60% of kilocalories from fat), and leptin‐deficient ob/ob mice were fed a ...
Keigo Tomoo, Yi Zhang, Ariana Zoe Diaz Portalatin, Gregory C. Henderson   +3 more
wiley   +1 more source

α1-Antitrypsin deficiency. [PDF]

, 2016
α1-Antitrypsin deficiency (A1ATD) is an inherited disorder caused by mutations in SERPINA1, leading to liver and lung disease. It is not a rare disorder but frequently goes underdiagnosed or misdiagnosed as asthma, chronic obstructive pulmonary disease ...
Brantly, Mark L, Ferrarotti, Ilaria, Greene, Catherine M, Lomas, David A, Marciniak, Stefan J, McElvaney, Noel G, Stoller, James K, Teckman, Jeffrey   +7 more
core   +2 more sources

Novel Therapeutics for Chronic Obstructive Pulmonary Disease: From Empirical Bronchodilation to Precision Medicine

Respirology, EarlyView.
ABSTRACT Chronic obstructive pulmonary disease (COPD) remains the third leading cause of death worldwide, and conventional bronchodilator‐based therapies have limited efficacy in preventing exacerbations and disease progression. The 2024–2026 period represents a historic inflection point: three mechanistically distinct agents received Food and Drug ...
Naoya Fujino, Hisatoshi Sugiura
wiley   +1 more source

Practice and knowledge about diagnosis and treatment of alpha-1 antitrypsin deficiency in Spain and Portugal [PDF]

, 2016
BACKGROUND: Determining physicians’ awareness about alpha-1 antitrypsin (AAT) deficiency (AATD) may help to explain the discrepancy between the observed and expected number of patients diagnosed with this disease.
Cristina Esquinas, Esther Rodriguez, Marc Miravitlles, Maria Sucena, Miriam Barrecheguren, Sandra Fernandez   +5 more
core   +4 more sources