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Erratum for the Research Article "An orthogonally regulatable DNA nanodevice for spatiotemporally controlled biorecognition and tumor treatment" by Z. Di <i>et al</i>.

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Pi-Z Phenotypes in a Pulmonary Clinic: Their Prevalence and Physiologic State

American Journal of Clinical Pathology, 1978
A series of 1,458 consecutive patients referred to the Cleveland Veterans Administration Pulmonary Clinic for pulmonary function studies was evaluated for alpha 1-antitrypsin deficiency by determination of serum trypsin inhibitory capacity (STIC). Protease inhibitor (Pi) phenotyping was performed on all sera with STIC values less than 1.6 mg/ml.
A A, Gerblich, J, Kleinerman, D J, Rynbrandt, E H, Chester, J, Ihrig   +4 more
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Age of SERPINA1 Gene PI Z Mutation: Swedish and Latvian Population Analysis

Annals of Human Genetics, 2008
SummaryAlpha 1‐antitrypsin (A1AT) deficiency, one of the most common inborn errors of metabolism in Caucasians, is characterized by a low serum concentration of A1AT and a high risk of pulmonary emphysema and liver disease. The allelic frequency for the most common protease inhibitor (PI) Z mutation in the SERPINA1 gene is 2–5% in Caucasians of ...
B, Lace, T, Sveger, A, Krams, G, Cernevska, A, Krumina   +4 more
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Occupational Exposure Risks in Individuals with PI*Z α₁-Antitrypsin Deficiency

American Journal of Respiratory and Critical Care Medicine, 2000
We obtained questionnaire and spirometry data from 128 alpha(1)-antitrypsin (alpha(1)AT)-deficient individuals with phenotype PI*Z to examine the relationship between chronic respiratory symptoms, airflow limitation, treatment requirements, and semiquantitative estimates of occupational exposure to dust, fumes, smoke, and gas.
A S, Mayer, J K, Stoller, B, Bucher Bartelson, A, James Ruttenber, R A, Sandhaus, L S, Newman   +5 more
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Pi*Z heterozygous alpha-1 antitrypsin states accelerate parenchymal but not biliary cirrhosis

European Journal of Gastroenterology & Hepatology, 2014
The degree to which heterozygous forms of alpha-1 antitrypsin (A1AT), principally MZ, causes liver disease is uncertain. If heterozygosity is a relevant cofactor, over-representation in patients with end-stage liver disease would be predicted. We therefore assessed the prevalence and disease-related distribution of A1AT heterozygosity in the largest ...
Tessa M, Cacciottolo, William T H, Gelson, Gerald, Maguire, Susan E, Davies, William J H, Griffiths   +4 more
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An Enzyme-Linked Immunosorbent Assay (ELISA) for Quantification of Circulating Pi*Z Alpha1-Antitrypsin Polymers

2023
Enzyme-linked immunosorbent assay (ELISA) is a sensitive immunoassay based on specific antigen-antibody reaction that is used for quantitative/qualitative analysis of various analytes in serum, plasma, saliva, cell and tissue lysates, and urine. ELISAs are typically performed in multi-well plates and depending on the design require coating antibody ...
Sabina, Janciauskiene, Tobias, Welte, Matthias, Lehmann   +2 more
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Liver Function in Asymptomatic Adult Individuals with Severe α₁-Antitrypsin Deficiency (Pi Z)

Scandinavian Journal of Gastroenterology, 1977
Eleven adult individuals (aged 24 to 66 years) with severe alpha1-antitrypsin deficiency, Pi Z, and with no clinical signs of liver disease, were investigated with a broad spectrum of liver function tests. Except for low alpha1-antitrypsin levels, no evidence of abnormal hepatic protein synthesis was found.
C, Larsson, S, Eriksson
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Alpha-1-Antitrypsin (PI) Polymorphism in France, with Special Regard to the PI*Z Allele

Human Heredity, 1991
Alpha-1-antitrypsin (PI) phenotypes were studied in a sample of more than 5,000 individuals from cities throughout France. Special interest was paid to the PI*Z allele whose average frequency, based on the present work plus results from the literature, was 0.0130. This figure was used to estimate the number of PI Z homozygotes in France.
R, Sesboüé, J P, Martin
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Natural History and Life Expectancy in Severe Alpha₁‐Antitrypsin Deficiency, Pi Z

Acta Medica Scandinavica, 1978
ABSTRACT. Clinical data from 246 adult Swedish individuals with severe α1‐antitrypsin deficiency, Pi Z, diagnosed in 1963‐77, were analyzed. Primary emphysema was present in 109 cases. Of 75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7 showed signs of emphysema.
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DNA restriction fragments associated with α1-antitrypsin indicate a single origin for deficiency allele PI Z

Nature, 1985
The alpha 1-protease inhibitor, or alpha-antitrypsin (AAT), a major plasma inhibitor of leukocyte elastase and bacterial proteases, is encoded at the PI locus on chromosome 14 (14q24.3-q32.1). A deficiency of AAT in individuals homozygous for the PI Z allele occurs in about 1 in 2,000-8,000 caucasians and is associated with an increased risk of early ...
D W, Cox, S L, Woo, T, Mansfield
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