... and that interactions of LMAN1 with an N-glycan of AAT is critical for this process. These results have implications in production of recombinant AAT and in developing treatments for AATD patients. cargo proteins cellular secretion endoplasmic reticulum intracellular transport serpin Glycoprotein α1...

...Neda Motamedi-Shad; Alistair M. Jagger; Maximilian Liedtke; Sarah V. Faull; Arjun Scott Nanda; Enrico Salvadori; Joshua L. Wort; Christopher W.M. Kay; Narinder Heyer-Chauhan; Elena Miranda; Juan Perez; Adriana Ordóñez; Imran Haq; James A. Irving; David A. Lomas Serpins are important regulators...

...Peter G.W. Gettins; Steven T. Olson Serpins are a widely distributed family of high molecular mass protein proteinase inhibitors that can inhibit both serine and cysteine proteinases by a remarkable mechanism-based kinetic trapping of an acyl or thioacyl enzyme intermediate that involves massive...

... mutations changing the P1′ residue or altering the reactive centre loop conformation significantly increased inhibition parameters, whereas removal of the positive charge within β-sheet C impeded the serpin–protease interaction. Arg 302 is a crucial contact to enable vaspin recognition by KLK7...

...James A. Irving; Imran Haq; Jennifer A. Dickens; Sarah V. Faull; David A. Lomas Serpins are protease inhibitors whose most stable state is achieved upon transition of a central 5-stranded β-sheet to a 6-stranded form. Mutations, low pH, denaturants and elevated temperatures promote this transition...

...Shoji Ishigami; Maria Sandkvist; Foon Tsui; Elizabeth Moore; Timothy A. Coleman; Daniel A. Lawrence Ns (neuroserpin) is a member of the serpin ( ser ine p rotease in hibitor) gene family that is primarily expressed within the central nervous system. Its principal target protease is tPA (tissue...

...Mareke Brüning; Martina Lummer; Caterina Bentele; Marcel M. W. Smolenaars; Kees W. Rodenburg; Hermann Ragg By alternative use of four RSL (reactive site loop) coding exon cassettes, the serpin (serine protease inhibitor) gene Spn4 from Drosophila melanogaster was proposed to enable the synthesis...

...Peter Stanley; Louise C. Serpell; Penelope E. Stein In the present study, we have investigated the in vitro polymerization of human plasma AGT (angiotensinogen), a non-inhibitory member of the serpin (SERine Protease INhibitor) family. Polymerization of AGT is thought to contribute to a high...

...Caterina Bentele; Olaf Krüger; Ulf Tödtmann; Mareke Oley; Hermann Ragg Lancelets are considered to take a key position in the evolution of lineages leading to vertebrates. Herein, a serpin from the lancelet Branchiostoma lanceolatum , Bl-Spn1, was identified that inhibits the PCs (proprotein...

... towards several macromolecular substrates, including gelatin, casein and albumin. Interestingly, activated matriptase-3 formed stable inhibitor complexes with an array of serpins, including plasminogen activator inhibitor-1, protein C inhibitor, α1-proteinase inhibitor, α2-antiplasmin and antithrombin III...

... an intracellular location and a wide distribution in bovine tissues. bovine muscle elastase inhibitor muscle endopin 1 serine proteinase serpin The trypsin/elastase inhibitor (mEndopin 1) was purified from bovine diaphragm muscle, obtained from the local slaughterhouse of the INRA Research Centre...

... ). 26 2 2003 1 5 2003 9 5 2003 9 5 2003 The Biochemical Society, London ©2003 2003 angiogenesis collagen-binding plasma pyroglutamate serpin Abbreviations used: BHK, baby-hamster kidney; CMM, calculated molecular mass; DTT, dithiothreitol; HRP, horseradish...

...] can inactivate the anti-proteolytic activity of the serpin plasminogen activator inhibitor-1 (PAI-1), a potential therapeutic target in cancer and cardiovascular diseases. Serpins inhibit their target proteases by the P 1 residue of their reactive centre loop (RCL) forming an ester bond...

... transition also had a retarded rate of polymerization. Studying a number of serpins by native gel electrophoresis, ligand-induced polymerization was observed only with PAI-1 and heparin cofactor II, which were also able to copolymerize. On the basis of these results, we suggest that the binding of ligands...

... 2002 14 6 2002 14 6 2002 The Biochemical Society, London ©2002 2002 apoptosis inhibitor of apoptosis protein (IAP) protease serpin Abbreviations used: Ac-DEVD-CHO, acetyl-Asp-Glu-Val-Asp-aldehyde; Ac-LEHD-AFC, acetyl-Leu-Glu-His-Asp-7-amino-4-trifluoromethylcoumarin...

...Rena M. HILL; Parmjeet K. PARMAR; Leigh C. COATES; Eva MEZEY; John F. PEARSON; Nigel P. BIRCH Two cDNAs encoding the serine protease inhibitor (serpin) neuroserpin were cloned from a rat pituitary cDNA library (rNS-1, 2922 bp; rNS-2, 1599 bp). In situ hybridization histochemistry showed neuroserpin...

...-binding protein), a specific serpin for this protease, as well as somatostatin 1–14. Internally quenched fluorogenic peptides were synthesized with the general structure Abz-peptidyl-EDDnp [Abz, o -aminobenzoic acid; EDDnp, N -(2,4-dinitrophenyl)ethylenediamine], based on the natural reactive-centre loop...