Identification of the major 4-methylumbelliferyl p-guanidinobenzoate-hydrolyzing plasma protein in cystic fibrosis: Implication for intrauterine and heterozygote detection

Bruce R. Branchini, Gino M. Salituro, Beryl J Rosenstein

Research output: Contribution to journalArticle

Abstract

Measurement of 4-methylumbelliferyIP-guanidinobenzoate (MUGB)-hydrolyzing activity in the plasma of normal controls, cystic fibrosis (CF) heterozygotes, and CF homozygotes did not support previously reported (35) differences in MUGB-hydrolyzing activity. We identified human plasma albumin as the major source of MUGB-hydrolyzing activity by comparison of our plasma results to those obtained with physiologic concentrations of commercial albumin samples. Substantiating evidence was obtained from gel filtration experiments and correlation of albumin levels in CF plasma with MUGB titers. We found essentially no proteolytic activity towards dinitrophenylprotamine sulfate associated with commercial albumin samples. It appears that the reaction between human albumin and MUGB represents a weak esterase activity, perhaps involving the acylation of a specific site(s) on the protein. Hypoalbuminemia has been documented (8) in some CF patients. Low albumin concentrations, indicated by MUGB titers

Original languageEnglish (US)
Pages (from-to)851-855
Number of pages5
JournalPediatric Research
Volume17
Issue number11
StatePublished - 1983

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Heterozygote Detection
Cystic Fibrosis
Blood Proteins
Albumins
Hypoalbuminemia
Acylation
Homozygote
Esterases
Heterozygote
Serum Albumin
Sulfates
Gel Chromatography
4-guanidinobenzoate

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

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title = "Identification of the major 4-methylumbelliferyl p-guanidinobenzoate-hydrolyzing plasma protein in cystic fibrosis: Implication for intrauterine and heterozygote detection",
abstract = "Measurement of 4-methylumbelliferyIP-guanidinobenzoate (MUGB)-hydrolyzing activity in the plasma of normal controls, cystic fibrosis (CF) heterozygotes, and CF homozygotes did not support previously reported (35) differences in MUGB-hydrolyzing activity. We identified human plasma albumin as the major source of MUGB-hydrolyzing activity by comparison of our plasma results to those obtained with physiologic concentrations of commercial albumin samples. Substantiating evidence was obtained from gel filtration experiments and correlation of albumin levels in CF plasma with MUGB titers. We found essentially no proteolytic activity towards dinitrophenylprotamine sulfate associated with commercial albumin samples. It appears that the reaction between human albumin and MUGB represents a weak esterase activity, perhaps involving the acylation of a specific site(s) on the protein. Hypoalbuminemia has been documented (8) in some CF patients. Low albumin concentrations, indicated by MUGB titers",
author = "Branchini, {Bruce R.} and Salituro, {Gino M.} and Rosenstein, {Beryl J}",
year = "1983",
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pages = "851--855",
journal = "Pediatric Research",
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TY - JOUR

T1 - Identification of the major 4-methylumbelliferyl p-guanidinobenzoate-hydrolyzing plasma protein in cystic fibrosis

T2 - Implication for intrauterine and heterozygote detection

AU - Branchini, Bruce R.

AU - Salituro, Gino M.

AU - Rosenstein, Beryl J

PY - 1983

Y1 - 1983

N2 - Measurement of 4-methylumbelliferyIP-guanidinobenzoate (MUGB)-hydrolyzing activity in the plasma of normal controls, cystic fibrosis (CF) heterozygotes, and CF homozygotes did not support previously reported (35) differences in MUGB-hydrolyzing activity. We identified human plasma albumin as the major source of MUGB-hydrolyzing activity by comparison of our plasma results to those obtained with physiologic concentrations of commercial albumin samples. Substantiating evidence was obtained from gel filtration experiments and correlation of albumin levels in CF plasma with MUGB titers. We found essentially no proteolytic activity towards dinitrophenylprotamine sulfate associated with commercial albumin samples. It appears that the reaction between human albumin and MUGB represents a weak esterase activity, perhaps involving the acylation of a specific site(s) on the protein. Hypoalbuminemia has been documented (8) in some CF patients. Low albumin concentrations, indicated by MUGB titers

AB - Measurement of 4-methylumbelliferyIP-guanidinobenzoate (MUGB)-hydrolyzing activity in the plasma of normal controls, cystic fibrosis (CF) heterozygotes, and CF homozygotes did not support previously reported (35) differences in MUGB-hydrolyzing activity. We identified human plasma albumin as the major source of MUGB-hydrolyzing activity by comparison of our plasma results to those obtained with physiologic concentrations of commercial albumin samples. Substantiating evidence was obtained from gel filtration experiments and correlation of albumin levels in CF plasma with MUGB titers. We found essentially no proteolytic activity towards dinitrophenylprotamine sulfate associated with commercial albumin samples. It appears that the reaction between human albumin and MUGB represents a weak esterase activity, perhaps involving the acylation of a specific site(s) on the protein. Hypoalbuminemia has been documented (8) in some CF patients. Low albumin concentrations, indicated by MUGB titers

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