Alpha-1-antitrypsin deficiency

DW Cox - Pediatric Liver Disease, 1983 - Springer
Abstract Alpha-1-antitrypsin (α 1 AT) is one of several protease inhibitors in serum which
together control proteases involved in such vital processes as activation of the clotting …

Molecular basis of alpha-1-antitrypsin deficiency

M Brantly, T Nukiwa, RG Crystal - The American journal of medicine, 1988 - Elsevier
Alpha-1-antitrypsin (A1AT) deficiency is an autosomal hereditary disorder associated with a
major reduction in serum A1AT levels. Clinically, A1AT deficiency is associated with …

Alpha-1-antitrypsin deficiency liver disease

D Patel, JH Teckman - Clinics in liver disease, 2018 - liver.theclinics.com
The liver is the primary site of synthesis of alpha-1-antitrypsin (AAT) protein, although it is
also made in enterocytes and some mononuclear white blood cells. 1 Large quantities of …

Alpha-1-antitrypsin deficiency: diagnosis and treatment

DH Perlmutter - Clinics in liver disease, 2004 - liver.theclinics.com
Alpha-1-antitrypsin (AT) deficiency was first described in the late 1960s by Laurell and
Eriksson [1] in patients with severe pulmonary emphysema. The recognition of AT deficiency …

Alpha-1-antitrypsin deficiency

HL Sharp - Hospital Practice, 1971 - Taylor & Francis
Two disparate disease states—an adult form of emphysema and a childhood form of liver
cirrhosis—have been linked to an inherited absence of this antienzyme. In their severe …

α1-antitrypsin deficiency: biological answers to clinical questions

RJ Coakley, C Taggart, S O'Neill… - The American journal of …, 2001 - Elsevier
ABSTRACT α1-antitrypsin (α1AT) deficiency is a common lethal hereditary disorder of white
persons of European descent. The condition is characterized by reduced serum levels of α …

α1-Antitrypsin deficiency: best clinical practice

NA Kalsheker - Journal of clinical pathology, 2009 - jcp.bmj.com
α1-Antitrypsin (AAT), a 52 kDa plasma protein, is produced mainly in the liver. It is the most
abundant circulating ser ine p roteinase in hibitor (serpin). It has also previously been called …

The current status of α-1-antitrypsin, a protease inhibitor, in gastrointestinal disease

HL Sharp - Gastroenterology, 1976 - Elsevier
Extremely deficient levels of α-1-antitrypsin (α1AT) predispose such deficientindividuals to
the development of emphysema and cirrhosis. Protease inhibitor (Pi) typing has clarified that …

α-1-Antitrypsin deficiency in liver disease: The extent of the problem

RL Fisher, L Taylor, S Sherlock - Gastroenterology, 1976 - Elsevier
A prospective screening program was undertaken at the Royal Free Hospital, London, to
ascertain the incidence of α-1-antitrypsin (AAT) deficiency in patients with liver disease …

Alpha-1-antitrypsin: an ignored protein in understanding liver disease

HL Sharp - Seminars in Liver Disease, 1982 - thieme-connect.com
Alpha-I-antitrypsin (oilAT) is an acute phase reacting a1 globulin synthesized by cells (most
notably the hepatocyte) for protection against tissue injury. This response is assumed to …