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Science 7 January 1972:
Vol. 175. no. 4017, pp. 63 - 65
DOI: 10.1126/science.175.4017.63
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Articles

Alpha1 Antitrypsin in the Livers of Patients with Emphysema

Jack Lieberman 1, Charles Mittman 1, and Herschel W. Gordon 1

1 Departments of Respiratory Diseases and Anatomic Pathology, City of Hope National Medical Center, Duarte, California 91010

Parenchymal liver cells from emphysema patients with an inherited deficiency of agr1-antitrypsin contain globules of glycoprotein that bind fluorescent antibody to agr1-antitrypsin. The globules can be seen after hematoxylin and eosinstaining or on electron microscopy, but are more readily demonstrated by PAS stain of amylase-treated liver sections. It appears that an inappropriately large amount of agr1-antitrypsin is found in the liver even when there is a deficiency in the serum. Genetic variants of the normal antitrypsin molecule may be unable to leave their site of synthesis in the liver cell because of some molecular aberration.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Alpha1-antitrypsin Deficiency and Severe Infantile Liver Disease.
P. ROSENTHAL, W. M. LIEBMAN, and M. M. THALER (1979)
Arch Pediatr Adolesc Med 133, 1195-1196
   Abstract »    PDF »
alpha1-Antitrypsin: the presence of excess mannose in the Z variant isolated from liver.
A Hercz, E Katona, E Cutz, J. Wilson, and M Barton (1978)
Science 201, 1229-1232
   Abstract »    PDF »
{alpha}1-Antitrypsin Deficiency Associated With Persistent Cutaneous Vasculitis: Occurrence in a Child With Liver Disease.
F. Brandrup and P. Aa. Ostergaard (1978)
Arch Dermatol 114, 921-924
   Abstract »    PDF »
{alpha}1-Antitrypsin Deficiency and Liver Disease in Children.
J. A. Burke, J. L. Kiesel, and J. D. Blair (1976)
Arch Pediatr Adolesc Med 130, 621-629
   Abstract »    PDF »
Pulmonary Emphysema and Liver Disease: Occurrence in an {alpha}1-Antitrypsin-Deficient Adult.
C. J. Donlan Jr., D. G. Ross, M. Golembieski, and J. V. Scutero (1975)
JAMA 232, 1147-1149
   Abstract »    PDF »
{alpha}1-Antitrypsin Deficiency, Emphysema, Cirrhosis, and Intestinal Mucosal Atrophy.
A. J. Greenwald, D. S. Johnson, R. M. Oskvig, C. A. Aschenbrener, and D. C. Randa (1975)
JAMA 231, 273-276
   Abstract »    PDF »
Hepatocellular Carcinoma and Partial Deficiency of Alpha-1 Antitrypsin (MZ).
W. RAWLINGS Jr., J. MOSS, H. S. COOPER, and S. R. HAMILTON (1974)
Ann Intern Med 81, 771-773
   Abstract »    PDF »
Infantile Cirrhosis With Hereditary Alpha1-Antitrypsin Deficiency: Clinical Improvement in Two Siblings.
R. C. Talamo and M. Feingold (1973)
Arch Pediatr Adolesc Med 125, 845-847
   Abstract »    PDF »
Alpha-1 Antitrypsin Deficiency, Emphysema, and Cirrhosis in an Adult.
K. L. COHEN, P. E. RUBIN, R. A. ECHEVARRIA, H. L. SHARP, and P. O. TEAGUE (1973)
Ann Intern Med 78, 227-232
   Abstract »    PDF »
Cirrhosis Associated with Partial Deficiency of Alpha-1 Antitrypsin in an Adult.
J. L. CAMPRA, J. R. CRAIG, R. L. PETERS, and T. B. REYNOLDS (1973)
Ann Intern Med 78, 233-238
   Abstract »    PDF »


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Science. ISSN 0036-8075 (print), 1095-9203 (online)