Author(s): Carrell RW
The archetypal status of alpha(1)-antitrypsin in biology and medicine grew from the finding, thirty years ago, by Carl-Bertil Laurell, of the association of its deficiency with emphysema. In biology, alpha(1)-antitrypsin now provides the model for both the structure and the remarkable mechanism of the serpin protease inhibitors that control the key proteolytic pathways of the body. In medicine, the plasma deficiency of alpha(1)-antitrypsin has drawn attention to protease-antiprotease imbalance as a contributory cause of chronic obstructive pulmonary disease. But even more significantly, the finding that the common genetic deficiency of alpha(1)-antitrypsin was also associated with the development of liver cirrhosis introduced the new entity of the conformational diseases. The proposal that the same general mechanism was responsible for the best known of the conformational diseases, the common late-onset dementias, was controversial. It was vindicated however by the recent finding that a mutation, which results in the liver aggregation of alpha(1)-antitrypsin, also results in a typical late-onset dementia when it occurs in a brain-specific homologue of alpha(1)-antitrypsin. The extensive development of such diverse fields of studies, each based on alpha(1)-antitrypsin, is a measure of the encouragement Laurell gave to younger colleagues in the field. It also reflects the great advantage of linked contributions from clinical as well as basic sciences. Time after time, scientific controversies and deadlocks have been solved by landmark clinical cases, which have revealed unexpected findings and insights, within and beyond the fields of study.
Referred From: https://www.ncbi.nlm.nih.gov/pubmed/16997740
Author(s): Abbott OA, Hopkins WA, Van Fleit WE , Robinson JS
Author(s): Barnes PJ
Author(s): Barnes PJ , Shapiro SD, Pauwels RA
Author(s): Barnes PJ
Author(s): Bast A , Haenen GR, Doelman CJ
Author(s): Bianco P, Robey P G
Author(s): BittmannI , Dose T, Baretton GB, Müller C, Schwaiblmair M, et al.
Author(s): Böyum A
Author(s): Brantigan O C, Mueller E, Kress M B
Author(s): Cooper JD, Patterson GA, Grossman R, Maurer J
Author(s): Cooper JD, Trulock EP, Triantafillou AN, Patterson GA, Pohl MS, et al.
Author(s): Dallegri F, Ottonello L
Author(s): Di Stefano A, Maestrelli P, Roggeri A, Turato G, Calabro S, et al.
Author(s): Di Stefano A, Capelli A, Lusuardi M, Balbo P, Vecchio C, et al.
Author(s): Eriksson S
Author(s): Holden C, Vogel G
Author(s): Huang Q, Liu D, Majewski P, Schulte LC, Korn JM, et al.
Author(s): Hyatt RE1, Farkas G, Schroeder M
Author(s): Ishizawa K, Kubo H, Yamada M, Kobayashi S, Numasaki M et al.
Author(s): Koca E, Champlin RE
Author(s): Kotton DN, Ma BY, Cardoso WV, Sanderson EA, Summer RS, et al.
Author(s): Lama VN , Smith L, Badri L, Flint A, Andrei AC, et al.
Author(s): Hogg J C, Walker B A
Author(s): Nikula KJ , March TH, Seagrave J, Finch G, Barr E, et al.
Author(s): Orlic D, Kajstura J, Chimenti S, Limana F, Jakoniuk I, et al.
Author(s): Prockop DJ
Author(s): Lagasse E , Connors H, Al-Dhalimy M, Reitsma M, Dohse M, et al.
Author(s): Krause DS, Theise ND, Collector MI, Henegariu O, Hwang S, et al.
Author(s): Finkelstein R, Fraser RS, Ghezzo H, Cosio MG
Author(s): Hellermann G R, Nagy S B, Kong, X.; Lockey, R.F.Mohapatra, S.S.
Author(s): Lopez AD, Murray CC
Author(s): Mahadeva R , Shapiro SD
Author(s): Martin GR
Author(s): Pushpakon R, Hogg J C, Woolcock A J, Angus A E, Macklem P T et al.
Author(s): Retamales I , Elliott WM, Meshi B, Coxson HO, Pare PD, et al.
Author(s): Russell RE , Thorley A, Culpitt SV, Dodd S, Donnelly LE, et al.
Author(s): Gordon B
Author(s): Gross P, Pfitzer E A, Tolker E, Babyak M A, Kaschak M
Author(s): Saetta M, Di Stefano A, Turato G, Facchini FM, Corbino L, et al.
Author(s): Saetta M, Turato G, Maestrelli P, Mapp CE, Fabbri LM
Author(s): Schrepfer S, Deuse T, Reichenspurner H, Fischbein MP, Robbins RC, et al.
Author(s): Senior R M, Anthonisen N R
Author(s): Shapiro SD
Author(s): Suratt BT, Cool CD, Serls AE, Chen L, Varella-Garcia M, et al.
Author(s): Takubo Y, Guerassimov A, Ghezzo H, Triantafillopoulos A, Bates JH, et al.
Author(s): Till JE, McCulloch EA
Author(s): Turhan AG
Author(s): Van Bekkum DW
Author(s): Knudson R J, Gaensler E A
Author(s): Wagers AJ, Weissman IL
Author(s): Wagner J G, Roth RA
Author(s): Yamada M, Kubo H, Kobayashi S, Ishizawa K, Numasaki M, et al.
Author(s): Xu J, Qu J, Cao L, Sai Y, Chen C, et al.
Author(s): Vidal R, Blanco I, Casas F, Jardí R, Miravitlles M; Committee on the National Registry of Individuals with Alpha-1 Antitrypsin Deficiency
Author(s): Costa X, Jardi R, Rodriguez F, Miravitlles M, Cotrina M, et al.
Author(s): Stoller JK, Aboussouan LS
Author(s): Seixas S, Garcia O, Trovoada MJ, Santos MT, Amorim A, et al.
Author(s): de Serres FJ
Author(s): Menezes AM, Perez-Padilla R, Jardim JR, Muiño A, Lopez MV, et al.
Author(s): de Faria EJ, de Faria IC, Alvarez AE, Ribeiro JD, Ribeiro AF, et al.
Author(s): Cruz PE, Mueller C, Cossette TL, Golant A, Tang Q, et al.
Author(s): Stockley RA, Luisetti M, Miravitlles M, Piitulainen E, Fernandez P; Alpha One International Registry (AIR) group
Author(s): Martorana PA, van Even P, Gardi C, Lungarella G
Author(s): Minguell JJ, Erices A, Conget P
Author(s): Nakayama K
Author(s): Neuringer IP , Randell SH
Author(s): Russell RE, Culpitt SV, DeMatos C, Donnelly L, Smith M, et al.
Author(s): Lieberman J, Winter B, Sastre A
Author(s): Popescu LM, Faussone-Pellegrini MS
Author(s): Popescu LM, Manole CG, Gherghiceanu M, Ardelean A, Nicolescu MI, et al.
Author(s): Popescu LM, Manole E, Serboiu CS, Manole CG, Suciu LC, et al.
Author(s): Mandache E, Gherghiceanu M, Macarie C, Kostin S, Popescu LM
Author(s): Gherghiceanu M, Popescu LM
Author(s): Cantarero I, Luesma MJ, Junquera C
Author(s): Cantarero Carmona I, Luesma Bartolomé MJ, Junquera Escribano C
Author(s): Zheng Y, Cretoiu D, Yan G, Cretoiu SM, Popescu LM, et al.
Author(s): Galiger C, Kostin S, Golec A, Ahlbrecht K, Becker S, et al.
Author(s): Zheng Y , Cretoiu D, Yan G, Cretoiu SM, Popescu LM, et al.
Author(s): Zheng Y Bai C, Wang X
Author(s): Popescu LM, Gherghiceanu M, Suciu LC, Manole CG, Hinescu ME
Author(s): Zheng Y , Li H, Manole CG, Sun A, Ge J, et al.
Author(s): Popescu LM, Gherghiceanu M, Suciu LC, Manole CG, Hinescu ME
Author(s): Hinescu ME, Gherghiceanu M, Suciu L, Popescu LM
Author(s): Suciu LC, Popescu BO, Kostin S, Popescu LM
Author(s): Díaz-Flores L, Gutiérrez R, García MP, Sáez FJ, Díaz-Flores L Jr, et al.
Author(s): Bani D, Formigli L, Gherghiceanu M, Faussone-Pellegrini MS
Author(s): Faussone-Pellegrini MS, Bani D
Author(s): Luesma MJ, Gherghiceanu M, Popescu LM
Author(s): Ceafalan L, Gherghiceanu M, Popescu LM, Simionescu O
Author(s): Manole C G, Cisma ÅŸiu V, Gherghiceanu M, Popescu LM