Morphological identification of alpha‐I‐antitrypsin in the human small intestine

Abstract

Alpha-I-antitrypsin immunoreactivity was demonstrated by immunofluorescence in epithelial cells of the normal human small intestine. Its presence was also confirmed in biopsies of patients with Crohn's disease. Specific fluorescence was observed in only four out of 14 adult patients with coeliac disease. These results implicate the human small intestinal epithelium as a possible source of alpha-I-antitrypsin. The absence of positive cells may have implications in the aetiology of coeliac disease.

Recombinant adeno-associated virus-mediated alpha-1 antitrypsin gene therapy prevents type I diabetes in NOD mice

“…9 AAT is primarily synthesized in the liver, but can also be produced by extrahepatic cells, including pancreatic islets, neutrophils, monocytes, macrophages, alveolar macrophages, intestinal epithelial cells, carcinoma cells and the cornea. [10][11][12][13][14][15] Normal serum levels of AAT in humans are 11-30 mM (0.8-2.4 mg/ml). During inflammation, infection and malignant diseases, AAT levels, as an acute phase reactant, can rise by 3-to 4-fold.…”

Antimicrobial Peptides in Saliva and Salivary Glands: Their Roles in the Oral Defense System

“…They are expressed in wound healing processes after tissue damage as well as in tissue remodeling. Alpha-ACT and -AT are found in many tumors and body fluids such as saliva, tears, milk, amniotic fluid and semen, as well as duodenal bile (194)(195)(196)(197)(198)(199)(200)(201). Human salivary glands are reactive to 1 -AT in ductal segments but unreactive to -ACT in glandular components (202).…”

Gene Therapy for Alpha-1-Antitrypsin Deficiency Diseases

“…The accumulated polymerized protein is retained in the periportal cells of the liver as diastaseresistant periodicacid/ schiff positive inclusions. The polymerized insoluble protein present in the endoplasmic reticulum of hepatocytes eventually causes neonatal hepatitis, juvenile cirrhosis and hepatocellular carcinoma (Geboes et al, 1982;Perlmutter et al, 1985;Ray et al, 1977). Owing to the polymerization in the ER of hepatocytes, the AAT is not secreted into the blood stream therefore the levels of circulating AAT in plasma decrease to <11 μM or <570 μg/ml and are unable to protect the lungs from proteolytic degradation.…”