Alpha-1 antitrypsin deficiency and recombinant protein sources with focus on plant sources: Updates, challenges and perspectives.

Alpha-1 antitrypsin deficiency (A1ATD) is an autosomal recessive disease characterized by low plasma levels of A1AT, a serine protease inhibitor representing the most abundant circulating antiprotease normally present at plasma levels of 1-2 g/L. The dominant clinical manifestations include predispositions to early onset emphysema due to protease/antiprotease imbalance in distal lung parenchyma and liver disease largely due to unsecreted polymerized accumulations of misfolded mutant A1AT within the endoplasmic reticulum of hepatocytes. Since 1987, the only FDA licensed specific therapy for the emphysema component has been infusions of A1AT purified from pooled human plasma at the 2020 cost of up to US $200,000/year with the risk of intermittent shortages.

An oxidation-resistant, recombinant alpha-1 antitrypsin produced in Nicotiana benthamiana.

Proteases and reactive oxygen species (ROS) have long been implicated in playing key roles in host tissue injury at sites of inflammation dominated by macrophage activations and/or neutrophil infiltrations. Imbalances between proteases/antiproteases and ROS/antioxidants are recognized to contribute to amplification of inflammatory-based host tissue injury. This has been especially well-documented in such respiratory tract diseases as chronic obstructive pulmonary disease, cystic fibrosis, and acute respiratory distress syndrome.

The Mother-Infant Feeding Relationship Across the First Year and the Development of Feeding Difficulties in Low-Risk Premature Infants.

Although feeding problems are common during infancy and are typically accompanied by relational difficulties, little research observed the mother-infant feeding relationship across the first year as an antecedent to the development of feeding difficulties. We followed 76 low-risk premature infants and their mothers from the transition to oral feeding in the neonatal period to the end of the first year. Prior to hospital discharge, microlevel patterns of maternal touch and gaze were coded during feeding and nonfeeding interactions, global patterns of maternal adaptation were assessed, and infants' neurobehavioral status was tested.