Results 71 to 80 of about 24,588 (237)

All-atom simulations reveal how single point mutations promote serpin misfolding

, 2018
Protein misfolding is implicated in many diseases, including the serpinopathies. For the canonical inhibitory serpin {\alpha}1-antitrypsin (A1AT), mutations can result in protein deficiencies leading to lung disease, and misfolded mutants can accumulate ...
Beccara, Silvio a, Faccioli, Pietro, Gershenson, Anne, Ianeselli, Alan, Orioli, Simone, Spagnolli, Giovanni, Wang, Fang, Wintrode, Patrick L.   +7 more
core   +1 more source

Distinct emphysema subtypes defined by quantitative CT analysis are associated with specific pulmonary matrix metalloproteinases. [PDF]

, 2016
BACKGROUND: Emphysema is characterised by distinct pathological sub-types, but little is known about the divergent underlying aetiology. Matrix-metalloproteinases (MMPs) are proteolytic enzymes that can degrade the extracellular matrix and have been ...
Bourne, S, Coombs, NA, Elkington, PT, Estepar, RS, Harden, S, Kim, V, Ostridge, K, Staples, KJ, Washko, G, Wilkinson, TM, Williams, N   +10 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

Alpha-1-antitrypsin deficiency and liver disease.

Acta Médica Portuguesa, 1981
In this review article only the liver disease associated with aipha-1-antitrypsin deficiency will be considered. For the well recognised association with lung disease the review by Mittman is recommended.
G. H. Millward-Sadler
doaj   +1 more source

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

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

Liver replacement for alpha1-antitrypsin deficiency [PDF]

, 1977
A 16-year-old girl with advanced cirrhosis and severe alpha1-antitrypsin deficiency of the homozygous PiZZ phenotype was treated by orthotopic liver transplantation.
Ashcavai, M, Peters, RL, Porter, KA, Putnam, CW, Redeker, AG, Starzl, TE   +5 more
core  

Assessing Major Adverse Liver Outcomes With Baclofen Compared to Acamprosate in Compensated Alcohol‐Associated Cirrhosis

Alimentary Pharmacology &Therapeutics, EarlyView.
We conducted a nationwide, multicenter cohort study using a target trial emulation framework to compare baclofen and acamprosate in patients with compensated alcohol‐associated cirrhosis. After matching, baclofen initiation was associated with a higher 1‐year risk of major adverse liver outcomes, driven primarily by hepatic encephalopathy, particularly
Yee Hui Yeo, Sepideh Mehravar, Ellen Choi, Ju Dong Yang, Hyun‐Seok Kim, Yun Wang, Aarshi Vipani, Hirsh D. Trivedi, Walid Ayoub, Parnian Naji, Alexander Kuo, Chun‐Ying Wu, Lorenzo Leggio, Juan Pablo Arab   +13 more
wiley   +1 more source

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  

Affinity Proteomics‐Based Non‐Invasive Detection of Clinically Significant Liver Disease

Alimentary Pharmacology &Therapeutics, EarlyView.
Using UK Biobank proteomic data, we identified a five‐protein score reflecting hepatic stellate cell activation and hepatocellular injury that predicts major adverse liver outcomes and clinically significant fibrosis, with consistent performance validated in two independent cohorts (patients with HIV and alpha1‐antitrypsin deficiency).
Sriram Balasubramani, Katharina Remih, Anna Sophie Karl, Julia Alexandra Borchert, Christina Schrader, Malin Fromme, Can Kayatekin, Bailin Zhang, Mikhail Levit, Pavithra Krishnaswami, Louise E. van Eekeren, Leo A. B. Joosten, Twan Otten, Petra Tomanová, Pavel Strnad   +14 more
wiley   +1 more source