Human urinary kallikrein was inhibited by human alpha 1-antitrypsin (alpha 1-protease inhibitor) in a similar way as by equivalent amounts of human serum. The inhibitor present in the kallikrein-inhibitor complex formed was identified as alpha 1-antitrypsin by two-dimensional immunoelectrophoresis. Under the experimental conditions applied, 90 mIU (= 185 microgram) alpha 1-antitrypsin inhibits about 9 microgram of human urinary kallikrein in 24 h at 37 degrees C, 1 ml of human serum, containing 2-4 mg alpha 1-antitrypsin, inhibits about 70 microgram kallikrein. At an incubation temperature of 25 degrees C, the rate of inactivation is significantly lower than at 37 degrees C. No inhibition was observed at 0 degrees C or when alpha 1-antitrypsin was presaturated with trypsin.
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http://dx.doi.org/10.1515/bchm2.1981.362.1.317 | DOI Listing |
Rev Physiol Biochem Pharmacol
January 2025
Institute of Medical Sciences, University of Aberdeen, Aberdeen, Scotland, UK.
Ribosomes use multiple electrical forces to regulate new protein construction, to ensure efficient protein cotranslation, chaperoning, and folding. When these electrical regulatory forces are disrupted as in point charge mutations, specific disease occurs from aberrantly folded proteins. α1 antitrypsin deficiency is perhaps the best-known misfolded protein disease and is covered in some detail.
View Article and Find Full Text PDFBiomedica
December 2024
Departamento de Medicina Interna, Facultad de Salud, Universidad del Valle, Cali, Colombia.
Introduction: Non-cystic fibrosis bronchiectasis is a complex medical condition with multiple etiologies, characterized by chronic productive cough and radiologic evidence of airway lumen dilation and wall thickening. Associated exacerbations and declining lung function contribute to increasing disability and mortality. There are no data about the prevalence of non-cystic fibrosis bronchiectasis etiologies in the Colombian population.
View Article and Find Full Text PDFRev Esp Enferm Dig
January 2025
Hepatology. Gastroenterology Unit, Hospital Universitario Central de Asturias.
A 16-year-old boy was diagnosed from multiple sclerosis (MS) after suffering from paresthesia in upper limbs and VI cranial nerve paresis. Corticosteroids and fingolimod were started. After 13 months a worsening of liver biochemical tests (LBT) was noticed: ALP 787 U/L, GGT 737 U/L, AST 195, ALT 321, Bi 0.
View Article and Find Full Text PDFPatient Relat Outcome Meas
January 2025
Institute of Applied Health Sciences, University of Birmingham, Birmingham, UK.
Alpha-1 antitrypsin deficiency (AATD) is a rare cause of chronic lung and liver disease without its own patient reported-outcome measure (PROM). PROMs for Chronic Obstructive Pulmonary Disease (COPD) are commonly used instead, but AATD differs from COPD in several ways. We reviewed whether the PROMs used in the AATD literature adequately assess quality-of-life in these patients.
View Article and Find Full Text PDFERJ Open Res
January 2025
Kamada Ltd., Rehovot, Israel.
Background: Alpha-1 antitrypsin (AAT)-deficient individuals have a greater risk for developing COPD than individuals with normal AAT levels.
Methods: This was a double-blind, randomised, parallel group, placebo-controlled trial to examine the safety and tolerability of "Kamada-AAT for Inhalation" (inhaled AAT) in subjects with AAT deficiency, and to explore its effect on AAT and biomarkers in the lung epithelial lining fluid (ELF). 36 patients with severe AAT deficiency were randomised 2:1 to receive 80 mg or 160 mg inhaled AAT or placebo once daily for 12 weeks.
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