RW Carrell, DA Lomas - The New England journal of medicine, 2002 - nejm.highwire.org Many biologic processes require a balance between the proteases that initiate
the proteolytic pathways essential to life and the inhibitors that limit
excessive protease activity. There are many different families of protease ... Cited by 247 - Related articles - BL Direct - All 12 versions
S Eriksson, I Hägerstrand - Acta Med Scand, 1974 - interscience.wiley.com Abstract. Clinical and laboratory findings in 9 adults with homozygous (Pi zz )
α 1 -antitrypsin (AAT) deficiency and cirrhosis of the liver are described.
This clinical entity is characterized by rapidly progressive cirrhosis in ... Cited by 91 - Related articles - All 2 versions
- ►nih.gov R Mahadeva, WSW Chang, TR Dafforn, DJ … - Journal of Clinical Investigation, 1999 - Am Soc Clin Investig The association between Z α 1 -antitrypsin deficiency and juvenile cirrhosis is
well-recognized, and there is now convincing evidence that the hepatic
inclusions are the result of entangled polymers of mutant Z α 1 ... Cited by 86 - Related articles - BL Direct - All 6 versions
- Free from Publisher D Lomas, H Parfrey - Thorax, 2004 - pubmedcentral.nih.gov The molecular basis of α 1 -antitrypsin deficiency is reviewed and is shown to
be due to the accumulation of mutant protein as ordered polymers within the
endoplasmic reticulum of hepatocytes. The current goals are to determine ... Cited by 61 - Related articles - BL Direct - All 5 versions
S Eriksson, T Moestrup, I Hägerstrand - Acta Med Scand, 1975 - interscience.wiley.com Abstract. The occurrence of PAS-positive, diastase-resistant, inclusion bodies
in hepatocytes has been used as a marker of heterozygous (Pi MZ) α 1
-antitrypsin deficiency in order to study the frequency of lung, liver and ... Cited by 55 - Related articles - All 2 versions
DR Triger, GH Millward-Sadler, AA … - QJM, 1976 - Oxford Univ Press SUMMARY Thirteen adult patients (aged 16 to 73 years) from 12 families are
described -with liver disease and alpha-1-antitrypsin deficiency. Long-term
observation of several of these patients suggests that the liver disease ... Cited by 32 - Related articles
- ►nih.gov [PDF] WK Blenkinsopp, GP Haffenden - British Medical Journal, 1977 - jcp.bmj.com Alpha-1-antitrypsin deficiency is associated with chronic liver disease in both
children and adults, and with emphysema in adults. The deficiency can be
identified by estimation of serum antitryptic activity, although ... Cited by 32 - Related articles - All 4 versions
- ►nih.gov [PDF] EJ Yunis, RM Agostini Jr, RH Glew - The American Journal of Pathology, 1976 - pubmedcentral.nih.gov Morphologic studies of liver tissue in individuals deficient in a-1-antitrvpsin
(ai-AT) have established the presence of membrane-delimited deposits which are
diastase resistant, periodic acid-Schiff positive, sialic acid deficient, ... Cited by 28 - Related articles - All 3 versions
