Methylumbelliferylguanidinobenzoate-reactive proteases in human amniotic fluid: Promising marker for the intrauterine detection of cystic fibrosis. 1980

M M Walsh, and H L Nadler

Over 1,000 specimens of midtrimester human amniotic fluid have been analyzed for 4-methylumbelliferylguanidinobenzoate (MUGB) reactivity. The mean specific activity value (nanomoles of methylumbelliferone (MU) formed per milligram of protein) for the control population (1,037) was 2.37 +/- 0.41. The mean specific activity values from a series of specimens of amniotic fluid derived from abnormal pregnancies (chromosomal and biochemical abnormalities, confirmed neural tube defects, abortions/stillbirths) were found not to vary significantly from value in the control population. The mean specific activity value of blood-tinged samples centrifuged to remove the red blood cell contaminants prior to freezing at -20 degrees C did not vary from the value for the control group, in contrast to the value for samples containing hemolyzed red blood cells (1.76 +/- 0.30). The mean specific activity values for all four groups (control, abnormal, spun and unspun) were found to be significantly different (p less than 0.001) from the mean specific activity value of 1.24 +/- 0.12 for the four cystic fibrosis (CF) amniotic fluids. In addition, upon isoelectric focusing of the control and CF fluids on polyacrylamide gels, pH 5-7, subsequently stained for alpha-N-benzoyl-L-arginine ethyl ester activity, the controls gave a pattern of five activity bands with a pl of 5.1 to 5.4, whereas the CF fluids consistently gave only four. These data suggest the potential value of the MUGB-reactive protease assay for the intrauterine detection of cystic fibrosis.

UI MeSH Term Description Entries
D010447 Peptide Hydrolases Hydrolases that specifically cleave the peptide bonds found in PROTEINS and PEPTIDES. Examples of sub-subclasses for this group include EXOPEPTIDASES and ENDOPEPTIDASES. Peptidase,Peptidases,Peptide Hydrolase,Protease,Proteases,Proteinase,Proteinases,Proteolytic Enzyme,Proteolytic Enzymes,Esteroproteases,Enzyme, Proteolytic,Hydrolase, Peptide
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D003550 Cystic Fibrosis An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006923 Hymecromone A coumarin derivative possessing properties as a spasmolytic, choleretic and light-protective agent. It is also used in ANALYTICAL CHEMISTRY TECHNIQUES for the determination of NITRIC ACID. Imecromone,Methylumbelliferone,Resocyanine,4-Methylumbelliferone,7-Hydroxy-4-methyl-coumarin,Cholestil,Mendiaxon,4 Methylumbelliferone,7 Hydroxy 4 methyl coumarin
D000649 Amniocentesis Percutaneous transabdominal puncture of the uterus during pregnancy to obtain amniotic fluid. It is commonly used for fetal karyotype determination in order to diagnose abnormal fetal conditions. Amniocenteses
D000653 Amniotic Fluid A clear, yellowish liquid that envelopes the FETUS inside the sac of AMNION. In the first trimester, it is likely a transudate of maternal or fetal plasma. In the second trimester, amniotic fluid derives primarily from fetal lung and kidney. Cells or substances in this fluid can be removed for prenatal diagnostic tests (AMNIOCENTESIS). Amniotic Fluid Index,Amniotic Fluid Indices,Amniotic Fluids,Fluid Index, Amniotic,Fluid Indices, Amniotic,Fluid, Amniotic,Fluids, Amniotic,Index, Amniotic Fluid,Indices, Amniotic Fluid
D014468 Umbelliferones 7-Hydroxycoumarins. Substances present in many plants, especially umbelliferae. Umbelliferones are used in sunscreen preparations and may be mutagenic. Their derivatives are used in liver therapy, as reagents, plant growth factors, sunscreens, insecticides, parasiticides, choleretics, spasmolytics, etc. 7-Hydroxycoumarins,Hydrangin,Skimmetin,7 Hydroxycoumarins

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