Circulating Truncated Alpha-1 Antitrypsin Glycoprotein in Patient Plasma Retains Anti-Inflammatory Capacity.


Journal

Journal of immunology (Baltimore, Md. : 1950)
ISSN: 1550-6606
Titre abrégé: J Immunol
Pays: United States
ID NLM: 2985117R

Informations de publication

Date de publication:
15 04 2019
Historique:
received: 27 07 2018
accepted: 30 01 2019
pubmed: 24 2 2019
medline: 28 12 2019
entrez: 24 2 2019
Statut: ppublish

Résumé

Alpha-1 antitrypsin (AAT) is an acute phase protein that possesses immune-regulatory and anti-inflammatory functions independent of antiprotease activity. AAT deficiency (AATD) is associated with early-onset emphysema and chronic obstructive pulmonary disease. Of interest are the AATD nonsense mutations (termed null or Q0), the majority of which arise from premature termination codons in the mRNA coding region. We have recently demonstrated that plasma from an AATD patient homozygous for the Null Bolton allele (

Identifiants

pubmed: 30796179
pii: jimmunol.1801045
doi: 10.4049/jimmunol.1801045
pmc: PMC6452030
mid: NIHMS1520672
doi:

Substances chimiques

Codon, Nonsense 0
SERPINA1 protein, human 0
alpha 1-Antitrypsin 0

Types de publication

Clinical Trial Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

2240-2253

Subventions

Organisme : NIDDK NIH HHS
ID : R01 DK101501
Pays : United States
Organisme : NHLBI NIH HHS
ID : R24 HL123828
Pays : United States
Organisme : NHLBI NIH HHS
ID : U01 HL134745
Pays : United States
Organisme : NCATS NIH HHS
ID : U01 TR001810
Pays : United States

Informations de copyright

Copyright © 2019 by The American Association of Immunologists, Inc.

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Auteurs

Emer P Reeves (EP)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland; emerreeves@rcsi.ie.

Danielle M Dunlea (DM)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.

Karen McQuillan (K)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.

Ciara A O'Dwyer (CA)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.

Tomás P Carroll (TP)

Alpha-1 Foundation Ireland, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, Ireland.

Radka Saldova (R)

GlycoScience Group, National Institute for Bioprocessing Research and Training, Mount Merrion, Dublin, Ireland.

Prithvi Reddy Akepati (PR)

Center for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118.

Mark R Wormald (MR)

Department of Biochemistry, Oxford Glycobiology Institute, University of Oxford, Oxford OX1 3QU, United Kingdom; and.

Oliver J McElvaney (OJ)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.

Vipatsorn Shutchaidat (V)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.

Michael Henry (M)

National Institute for Cellular Biotechnology, Dublin City University, Dublin 9, Ireland.

Paula Meleady (P)

National Institute for Cellular Biotechnology, Dublin City University, Dublin 9, Ireland.

Joanne Keenan (J)

National Institute for Cellular Biotechnology, Dublin City University, Dublin 9, Ireland.

Derek C Liberti (DC)

Center for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118.

Darrell N Kotton (DN)

Center for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118.

Pauline M Rudd (PM)

Alpha-1 Foundation Ireland, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, Ireland.

Andrew A Wilson (AA)

Center for Regenerative Medicine, Boston University and Boston Medical Center, Boston, MA 02118.

Noel G McElvaney (NG)

Irish Centre for Genetic Lung Disease, Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin 9, Ireland.

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Classifications MeSH