Figure 6.

Cela1 in human emphysema. (A) Immunohistochemistry for Cela1 in normal human lung revealed very few Cela1 protein–containing cells. (B) A specimen obtained from a patient without AAT deficiency (MM genotype) undergoing lung transplant revealed scattered Cela1 protein–containing cells throughout the alveolar interstitium. (C) Examination of a lung specimen from an individual with AAT deficiency (ZZ genotype) undergoing lung transplant identified Cela1 protein–containing cells in regions of airspace destruction. (D) Quantification of Cela1 protein–containing cells in control, AAT-sufficient (MM genotype), and AAT-deficient (ZZ genotype) patients identified a trend toward an increased number of Cela1-positive cells in AAT-sufficient and AAT-deficient emphysema. Only two of five control lungs had numbers of Cela1-containing cells above the number observed in secondary-alone control (dashed line). (E) A separate ZZ genotype AAT-deficient emphysema specimen with staining for the macrophage marker CD68 (green) and pro-SPB (red) human Cela1 mRNA by proximity ligation in situ hybridization (white) and DAPI (blue). Scattered Cela1 mRNA–containing cells are seen in this region of airspace destruction. E′ is a magnification of the boxed area in E demonstrating ATII cells with (white arrows) and without (red arrows) Cela1 mRNA. E″ displays magnification of only DAPI and Cela1 proximity ligation in situ hybridization signals. Scale bars: 100 μm (A, B, and C), 250 μm (E), and 10 μm (E′ and E").