Article
Pharmacology & Pharmacy
Anna Annunziata, Maurizia Lanza, Antonietta Coppola, Paolo Andreozzi, Sara Spinelli, Giuseppe Fiorentino
Summary: This article describes the experience of using home care intravenous augmentation therapy to manage human alpha-1 antitrypsin deficiency during the emergency of the SARS-CoV2 infection outbreak. The focus is on evaluating the safety of home treatment and the quality of life of patients enrolled in the program.
FRONTIERS IN PHARMACOLOGY
(2021)
Review
Dermatology
Alessandro N. Franciosi, James Ralph, Naoimh J. O'Farrell, Colm Buckley, Christian Gulmann, Marina O'Kane, Tomas P. Carroll, Noel G. McElvaney
Summary: The typical features and investigation of AATD-associated panniculitis need further clarification. Dapsone is the most cost-effective therapeutic option, while intravenous AAT augmentation therapy is the most efficacious.
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
(2022)
Article
Medicine, General & Internal
Leonard Riley, Aryaman Sriram, Mark Brantly, Jorge Lascano
Summary: This retrospective cohort study aimed to investigate the testing frequency and disparities for alpha-1 antitrypsin deficiency. The results showed that testing for AAT deficiency continues to have a low uptake in the clinical setting, although it is slowly improving. Individuals of White race and those with concomitant chronic obstructive pulmonary disease and liver disease are more likely to be tested.
AMERICAN JOURNAL OF MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Noor Ahmad Shaik, Najla Bint Saud Al-Saud, Thamer Abdulhamid Aljuhani, Kaiser Jamil, Huda Alnuman, Deema Aljeaid, Nasreen Sultana, Ashraf AbdulRahman El-Harouni, Zuhier Ahmed Awan, Ramu Elango, Babajan Banaganapalli
Summary: This study investigates the impact of SERPINA1 missense variants on the structural and functional characteristics of A1AT protein in Alpha-1 antitrypsin deficiency. The results suggest that these variants alter the structure, stability, and function of A1AT protein, and negatively affect its binding with NE ligand molecule.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2022)
Article
Biochemistry & Molecular Biology
Daniella A. Spittle, Alison Mansfield, Anita Pye, Alice M. Turner, Michael Newnham
Summary: The progression of lung disease in alpha-1 antitrypsin deficiency (AATD) varies and presents differently. Blood biomarkers are a convenient and repeatable method of monitoring diseases. In this study, we compared the levels of seven serum biomarkers between AATD patients with and without COPD. We found that CRP and CCL18 were significantly higher in AATD COPD patients. CC16 was found to be predictive of emphysema progression in AATD COPD.
Article
Medicine, Research & Experimental
Lena Ostermann, Regina Maus, Jennifer Stolper, Lisanne Schutte, Konstantina Katsarou, Srinu Tumpara, Andreas Pich, Christian Mueller, Sabina Janciauskiene, Tobias Welte, Ulrich A. Maus
Summary: The study found that in AAT-deficient mice, NE can degrade lung SP-A and SP-D, affecting lung protective immunity. Treatment with human AAT or the NE-specific inhibitor Sivelestat can protect collectins from degradation and reduce bacterial loads in S. pneumoniae-infected AAT-KO mice.
Article
Instruments & Instrumentation
Annalisa Bianchera, Viviana Vilardo, Roberta Giaccari, Annalisa Michielon, Gianluca Bazzoli, Francesca Buttini, Marina Aiello, Alfredo Chetta, Stefano Bruno, Ruggero Bettini
Summary: Two nebulizers, a jet and a mesh vibrating system, were compared in terms of performance and effectiveness for delivering alpha-1 antitrypsin (AAT) to the lungs. The mesh nebulizer demonstrated higher efficiency in drug delivery, with preserved protein activity and conformation. Nebulization of AAT is a suitable administration strategy for AATD patients, either as a support therapy or for preventive purposes.
DRUG DELIVERY AND TRANSLATIONAL RESEARCH
(2023)
Article
Gastroenterology & Hepatology
Christoph Grander, Benedikt Schaefer, Julian Schwaerzler, Felix Grabherr, Dennis M. de Graaf, Barbara Enrich, Georg Oberhuber, Lisa Mayr, Moris Sangineto, Nikolai Jaschke, Timon E. Adolph, Maria Effenberger, Alexander R. Moschen, Charles A. Dinarello, Heinz Zoller, Herbert Tilg
Summary: ALD is a global healthcare issue with limited treatment options. AAT has shown potent anti-inflammatory activities and low serum concentrations are associated with increased risk of death/liver transplantation in cirrhotic ALD patients. Experimental models in mice suggest that AAT may be a potential therapeutic option for ALD, especially for alcoholic hepatitis.
Article
Biochemistry & Molecular Biology
Magdalena K. Kaneva, Milind M. Muley, Eugene Krustev, Allison R. Reid, Patricia R. Souza, Francesco Dell'Accio, Jason J. McDougall, Mauro Perretti
Summary: The study demonstrates that AAT possesses anti-inflammatory, analgesic, and chondroprotective properties, reversing joint inflammation and cartilage degradation, promoting the transcription of cartilage-related genes, enhancing chondrogenic differentiation, and acting through CREB signaling pathway and inhibition of Wnt/beta-catenin pathways.
Article
Medical Laboratory Technology
Loris Wauthier, Stephanie Jacques, Joris Delanghe, Julien Favresse
Summary: This study compared two serum protein electrophoresis systems to detect decreased A1AT concentrations, and found that the V8 Nexus alpha(1) band 2 was the best predictor of A1AT deficiency.
CLINICAL CHEMISTRY AND LABORATORY MEDICINE
(2023)
Article
Biochemical Research Methods
Shelley Jager, Dario A. T. Cramer, Albert J. R. Heck
Summary: Alpha-1-antitrypsin (A1AT) has been suggested as a potential biomarker for distinguishing healthy and diseased individuals. However, the variability of the SERPINA1 gene in the general population may affect A1AT expression and serum protein levels, which are often overlooked in proteomics studies. This study found significant differences in allele-specific protein serum levels of A1AT among heterozygous donors, suggesting the importance of considering these factors when analyzing A1AT as a potential serum biomarker.
JOURNAL OF PROTEOME RESEARCH
(2023)
Article
Pharmacology & Pharmacy
Xiao Liu, Kevin Vanvarenberg, Kobenan Guy Wilfried Kouassi, Sohaib Mahri, Rita Vanbever
Summary: In this study, a PEGylated version of AAT was produced and characterized in vitro, showing potential for prolonged body residence time and improved therapeutic efficacy via intravenous and inhalation routes.
INTERNATIONAL JOURNAL OF PHARMACEUTICS
(2022)
Article
Cardiac & Cardiovascular Systems
Joanna Chorostowska-Wynimko, A. Rembert Koczulla, Maria Sucena
Summary: There are variations in the management of AATD in different European countries, highlighting the need for education and standardization. Familiarity with and use of ERN-LUNG AATD services vary, indicating a need for increased awareness of these services.
RESPIRATORY MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Kanoko Yoshida, Aruto Yano, Kazuya Kusama, Gen Ishikawa, Kazuhiro Tamura
Summary: The study suggests that A1AT may negatively regulate syncytialization in the placental trophoblasts by controlling inflammatory responses and the activation of p38MAPK and JNK pathways. Dysfunction of A1AT could be responsible for abnormal placental formation and pregnancy-associated disorders.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Multidisciplinary Sciences
Sangmi S. Park, Michelle Mai, Magdalena Ploszaj, Huchong Cai, Lucas McGarvey, Christian Mueller, Itsaso Garcia-Arcos, Patrick Geraghty
Summary: Type 1 diabetes is associated with an increased prevalence of pulmonary disease and can worsen the progression of lung damage in individuals with alpha-1 antitrypsin deficiency. This study found that T1D mice exhibited restrictive lung function patterns and increased expression of pro-fibrotic markers, while T1D in AAT-deficient mice led to collagen accumulation and enlarged alveolar spaces. AAT pretreatment was shown to reduce fibrotic marker expression in lung fibroblasts. Overall, this research highlights the importance of considering the interaction between T1D and AAT deficiency in the development of lung complications.
Article
Biotechnology & Applied Microbiology
Minerva Daya, Watcharin Loilome, Anchalee Techasen, Malinee Thanee, Prakasit Sa-Ngiamwibool, Attapol Titapun, Puangrat Yongvanit, Nisana Namwat
ONCOTARGETS AND THERAPY
(2018)
Article
Biotechnology & Applied Microbiology
Sasikamon Khophai, Malinee Thanee, Anchalee Techasen, Nisana Namwat, Poramate Klanrit, Attapol Titapun, Apiwat Jarearnrat, Prakasit Sa-Ngiamwibool, Watcharin Loilome
ONCOTARGETS AND THERAPY
(2018)
Review
Pathology
Kitti Intuyod, Napat Armartmuntree, Apinya Jusakul, Chadamas Sakonsinsiri, Raynoo Thanan, Somchai Pinlaor
EXPERT REVIEW OF MOLECULAR DIAGNOSTICS
(2019)
Article
Oncology
Ravinnipa Chanakankun, Tanakorn Proungvitaya, Daraporn Chua-On, Temduang Limpaiboon, Sittiruk Roytrakul, Apinya Jusakul, Attapol Titapun, Apiwat Jarearnrat, Siriporn Proungvitaya
Article
Multidisciplinary Sciences
Thatsanapong Pongking, Ornuma Haonon, Rungtiwa Dangtakot, Sudarat Onsurathum, Apinya Jusakul, Kitti Intuyod, Arunnee Sangka, Sirirat Anutrakulchai, Ubon Cha'on, Somchai Pinlaor, Porntip Pinlaor
Article
Multidisciplinary Sciences
Achira Namjan, Anchalee Techasen, Watcharin Loilome, Prakasit Sa-ngairnwibool, Apinya Jusakul
Article
Multidisciplinary Sciences
Laongthip Ruknarong, Chongchira Boonthongkaew, Nisa Chuangchot, Amonrat Jumnainsong, Naruemon Leelayuwat, Apinya Jusakul, Silvana Gaudieri, Chanvit Leelayuwat
Summary: The study found that vitamin C supplementation can alter the expression of circulating miR-451a in patients with T2DM, potentially serving as a biomarker for oxidative status and poor glycemic control, and leading to a novel molecular strategy to reduce oxidative stress in these patients.
Article
Oncology
Suyanee Thongchot, Chiara Vidoni, Alessandra Ferraresi, Watcharin Loilome, Narong Khuntikeo, Sakkarn Sangkhamanon, Attapol Titapun, Ciro Isidoro, Nisana Namwat
Summary: The study validates the hypothesis that CAF infiltration and IL-6 release predict poor prognosis in CCA patients, with low stromal IL-6 and active autophagy in cancer cells correlating with better outcomes and chemotherapy response. Therapeutic strategies targeting these factors may improve survival in CCA patients.
Article
Biochemistry & Molecular Biology
Bundit Promraksa, Praewpan Katrun, Jutarop Phetcharaburanin, Yingpinyapat Kittirat, Nisana Namwat, Anchalee Techasen, Jia V. Li, Watcharin Loilome
Summary: The study demonstrated that CA can induce apoptosis in CCA cells and may serve as a potent anticancer agent for CCA treatment. CA affects the half-inhibitory concentration of CCA cells and cellular metabolism, involving antioxidant defense mechanism, glycerophospholipid metabolism, and the tricarboxylic acid cycle.
Article
Medicine, General & Internal
Preawwalee Wintachai, Jing Quan Lim, Anchalee Techasen, Worachart Lert-Itthiporn, Sarinya Kongpetch, Watcharin Loilome, Jarin Chindaprasirt, Attapol Titapun, Nisana Namwat, Narong Khuntikeo, Apinya Jusakul
Summary: The study found that cfDNA levels were significantly higher in CCA compared to benign biliary disease and normal controls. cfDNA levels showed superior diagnostic efficacy in discriminating CCA from healthy controls and BBD.
Article
Multidisciplinary Sciences
Rujikorn Treeriya, Phuc N. Ho, Attapol Titapun, Poramate Klanrit, Manida Suksawat, Thanaporn Kulthawatsiri, Suphasarang Sirirattanakul, Watcharin Loilome, Nisana Namwat, Arporn Wangwiwatsin, Nittaya Chamadol, Narong Khuntikeo, Jutarop Phetcharaburanin
Summary: In this study, nuclear magnetic resonance (NMR) metabolomics was used to investigate the fecal metabolic phenotypes of different study groups, including normal bile duct, periductal fibrosis (PDF), and cholangiocarcinoma (CCA) patients. The results revealed distinct fecal metabolic patterns in PDF and CCA patients compared to the normal bile duct group, indicating the involvement of various metabolic pathways in PDF and CCA progression. The study also highlighted the role of gut-microbial host metabolic crosstalk in PDF and/or CCA patients.
Article
Oncology
Napat Armartmuntree, Apinya Jusakul, Chadamas Sakonsinsiri, Watcharin Loilome, Somchai Pinlaor, Piti Ungarreevittaya, Chern Han Yong, Anchalee Techasen, Kanokwan Imtawil, Ratthaphol Kraiklang, Nattawan Suwannakul, Waleeporn Kaewlert, Timpika Chaiprasert, Raynoo Thanan, Mariko Murata
Summary: The study demonstrates that DNA hypermethylation in the EBF1 promoter region suppresses EBF1 expression and induces CCA progression. Treatment with a DNA methyltransferase inhibitor leads to increased EBF1 expression levels and inhibition of cell growth, migration, and invasion in CCA cells. RNA sequencing analysis suggests that EBF1 is involved in suppression of multiple pathways in cancer.
Article
Oncology
Sarinya Kongpetch, Apinya Jusakul, Jing Quan Lim, Cedric Chuan Young Ng, Jason Yongsheng Chan, Vikneswari Rajasegaran, Tse Hui Lim, Kiat Hon Lim, Su Pin Choo, Simona Dima, Irinel Popescu, Dan G. Duda, Veerapol Kukongviriyapan, Narong Khuntikeo, Chawalit Pairojkul, Steven G. Rozen, Patrick Tan, Bin Tean Teh
JCO GLOBAL ONCOLOGY
(2020)
Article
Oncology
Phongsaran Kimawaha, Apinya Jusakul, Prem Junsawang, Watcharin Loilome, Narong Khuntikeo, Anchalee Techasen
JOURNAL OF GASTROINTESTINAL ONCOLOGY
(2020)