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Plasma levels of TIMP-1 are higher in 34-year-old individuals with severe α1-antitrypsin deficiency
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  1. Sabina Janciauskiene1,
  2. Devipriya Subramaniyam1,
  3. Eeva Piitulainen2,
  4. Thomas Köhnlein1,
  5. Tomas Sveger3
  1. 1Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany
  2. 2Department of Respiratory Medicine, Lund University, Institute of Clinical Sciences, Malmö University Hospital, Malmö, Sweden
  3. 3Department of Paediatrics, Lund University, Institute of Clinical Sciences, Malmö University Hospital, Malmö, Sweden
  1. Correspondence to Sabina Janciauskiene, Department of Respiratory Medicine, Hannover Medical School, Carl-Neuberg Str. 1, D-30625 Hannover, Germany; Janciauskiene.Sabina{at}mh-hannover.de

https://doi.org/10.1136/thx.2009.127936

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    α1-Antitrypsin (AAT) is one of the major proteins in the circulation eliciting significant protection against activated serine proteases, such as neutrophil elastase and proteinase-3. Moderate and severe AAT deficiency (AATD) is almost entirely caused by the Z (Glu342 to lysine change) and S (Glu264 to valine change) alleles as opposed to the normal wild-type M allele. Severe ZZ deficiency of AAT, characterised by a decrease in serum AAT levels to values <20% of normal, entails a high risk of developing pulmonary emphysema. This fact provides the rationale for the protease–antiprotease imbalance theory of the pathogenesis of emphysema.

    We hypothesised that in subjects with AATD, other protease inhibitors may mitigate the …

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