The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization

FEBS Journal

Volumn 284, Issue 13, 2017, Pages 2110-2126

  Miranda, Elena ^a   Ferrarotti, Ilaria ^b   Berardelli, Romina ^c   Laffranchi, Mattia ^c   Cerea, Marta ^a,c   Gangemi, Fabrizio ^c   Haq, Imran ^d   Ottaviani, Stefania ^e   Lomas, David A ^d   Irving, James A ^d   Fra, Annamaria ^c  

^a SAPIENZA UNIVERSITY OF ROME   (Italy)

^b UNIVERSITY OF PAVIA   (Italy)

^c UNIVERSITY OF BRESCIA   (Italy)

^d UNIVERSITY COLLEGE LONDON   (United Kingdom)

^e FONDAZIONE IRCCS POLICLINICO SAN MATTEO   (Italy)

Author keywords

alpha 1 antitrypsin deficiency; emphysema; misfolding diseases; serpin polymers; serpins

Indexed keywords

ALPHA 1 ANTITRYPSIN; ALPHA 1 ANTITRYPSIN TRENTO VARIANT; EPITOPE; GLUTAMIC ACID; MONOCLONAL ANTIBODY; OLIGOMER; POLYMER; PROTEIN VARIANT; UNCLASSIFIED DRUG; VALINE; SERPINA1 PROTEIN, HUMAN;

ARTICLE; CONTROLLED STUDY; ENZYME LINKED IMMUNOSORBENT ASSAY; HUMAN; HUMAN CELL; MUTANT; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; PROTEIN CONFORMATION; PROTEIN DEFECT; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN INTERACTION; PROTEIN POLYMERIZATION; PROTEIN SECRETION; PROTEIN STABILITY; PROTEIN STRUCTURE; ALPHA 1 ANTITRYPSIN DEFICIENCY; BLOOD; CASE REPORT; CHEMISTRY; GENETIC PREDISPOSITION; GENETICS; MALE; METABOLISM; MIDDLE AGED; MOLECULAR DYNAMICS; MUTATION; POLYMERIZATION;

ALPHA 1-ANTITRYPSIN; ALPHA 1-ANTITRYPSIN DEFICIENCY; ELECTROPHORESIS, POLYACRYLAMIDE GEL; ENZYME-LINKED IMMUNOSORBENT ASSAY; GENETIC PREDISPOSITION TO DISEASE; HUMANS; MALE; MIDDLE AGED; MOLECULAR DYNAMICS SIMULATION; MUTATION; POLYMERIZATION; PROTEIN CONFORMATION;

EID: 85020304986     PISSN: 1742464X     EISSN: 17424658     Source Type: Journal    
DOI: 10.1111/febs.14111     Document Type: Article

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