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May 1974, Vol 127, No. 5 >
Article | May 1974

Beta-Glucuronidase Activity in the Sweat of Children With Cystic Fibrosis FREE

Bruce W. Evans; Robert C. Kelsch, MD; William F. Howatt, MD
[+] Author Affiliations

Received for publication Aug 24, 1973; accepted Dec 17.

Reprint requests to Department of Pediatrics and Communicable Disease, University of Michigan, Ann Arbor, MI 48104 (Dr. Howatt).


Am J Dis Child. 1974;127(5):660-662. doi:10.1001/archpedi.1974.02110240046004.
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References
Comments

The activity of β-glucuronidase in the sweat of subjects with cystic fibrosis, known carriers of the disease, and normal subjects was determined by a spectrofluorometric method by means of 1-naphthol-B-D glucuronide as the substrate. In normal subjects, the enzyme activity was greater in sweat collected during the first five to ten minutes after stimulation, compared to that collected over the next 30 to 40 minutes. There was no substantial difference in enzyme activity of sweat collected during the 30 to 40 minutes after the washout period among normal, cystic fibrosis, and carrier subjects. The Km values of β-glucuronidase activity in sweat from normal and cystic fibrosis subjects were the same.

REFERENCES

1 +
Anderson WAD, Scotti TM:  Liver, gallbladder and pancreas , in Synopsis of Pathology , ed 8. St. Louis, CV Mosby Co, 1972;, pp 613-614.
2 +
Gibbs G, Griffin G:  Quantitative determination of beta-glucuronidase in sweat gland and other skin components in cystic fibrosis . J Invest Dermatol 51:200-203, 1968;.
3 +
Gibbs G, Griffin G:  Beta glucuronidase activity in skin components of children with cystic fibrosis . Science 167:993-994, 1970;.
4 +
Dorfman A, Matalon R:  The mucopolysaccharidoses , in Stanbury JB, Wyngaarden JB, Fredrickson DS (eds): The Metabolic Basis of Inherited Disease , ed 3. New York, McGraw-Hill Book Co, 1972;, pp 1218-1272.
5 +
Verity MA, Caper VR, Brown WJ:  Spectrofluorometric determination of β-glucuronidase activity . Arch Biochem Biophys 106:386-393, 1964;.
6 +
Lineweaver H, Burk D:  The determination of enzyme dissociation constants . Am Chem Soc 56:658-660, 1934;.
7 +
Lobeck CC, Huebner D:  Effect of age, sex, and cystic fibrosis on the sodium and potassium content of human sweat . Pediatrics 30:172-179, 1962;.
8 +
Wilson RG:  The activities of some acid hydrolases in the skin of patients with cystic fibrosis of the pancreas . Clin Chim Acta 36:113-117, 1972;.
9 +
Griffin GD, Gibbs GE:  Lysosomal enzymes of cultured white blood cells in cystic fibrosis . Proc Soc Exp Biol Med 137:438-442, 1971;.
10 +
Marchi AG, Mangiarotti Marchi MA, Nordio S:  Studio di attivita enzimatiche lisosomiali in linfociti fito-stimolati nella fibrosi cistica del pancreas . Minerva Pediatr 23:1842-1846, 1971;.
11 +
Antonowicz I, Sippell WG, Schwachman H:  Lysosomal and mitochondrial enzyme activities of lymphoid cell lines . Pediatr Res 6:803-812, 1972;.
12 +
Benke PJ:  Beta glucuronidase and cystic fibrosis . N Engl J Med 284:731-732, 1971;.
13 +
Knaus I, et al:  Metachromasia and assay for lysosomal enzymes in skin fibroblasts cultured from patients with cystic fibrosis and controls . Pediatrics 47:1010-1018, 1971;.
14 +
Butterworth J, et al:  Lysosomal enzymes of cultured fibroblasts of cystic fibrosis patients . Clin Chim Acta 40:139-142, 1972;.
15 +
Antonowicz I, Schwachman H:  Beta-galactosidase and beta-glucuronidase in intestinal mucosa . Pediatrics 47:737-744, 1971;.
16 +
Maxfield M, Wolins W:  A molecular abnormality of urinary mucoprotein in cystic fibrosis of the pancreas . J Clin Invest 41:455-462, 1972;.
17 +
Danes BS, Bearn AG:  Cystic fibrosis: Distribution of mucopolysaccharides in fibroblast cultures . Biochem Biophys Res Commun 36:919-924, 1969;.
18 +
Crosby P, Marsden JC, Robards AW:  Electron microscopy of urinary glycoproteins and its relation to cystic fibrosis . Histochem J 3:223-232, 1971;.

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References

1 +
Anderson WAD, Scotti TM:  Liver, gallbladder and pancreas , in Synopsis of Pathology , ed 8. St. Louis, CV Mosby Co, 1972;, pp 613-614.
2 +
Gibbs G, Griffin G:  Quantitative determination of beta-glucuronidase in sweat gland and other skin components in cystic fibrosis . J Invest Dermatol 51:200-203, 1968;.
3 +
Gibbs G, Griffin G:  Beta glucuronidase activity in skin components of children with cystic fibrosis . Science 167:993-994, 1970;.
4 +
Dorfman A, Matalon R:  The mucopolysaccharidoses , in Stanbury JB, Wyngaarden JB, Fredrickson DS (eds): The Metabolic Basis of Inherited Disease , ed 3. New York, McGraw-Hill Book Co, 1972;, pp 1218-1272.
5 +
Verity MA, Caper VR, Brown WJ:  Spectrofluorometric determination of β-glucuronidase activity . Arch Biochem Biophys 106:386-393, 1964;.
6 +
Lineweaver H, Burk D:  The determination of enzyme dissociation constants . Am Chem Soc 56:658-660, 1934;.
7 +
Lobeck CC, Huebner D:  Effect of age, sex, and cystic fibrosis on the sodium and potassium content of human sweat . Pediatrics 30:172-179, 1962;.
8 +
Wilson RG:  The activities of some acid hydrolases in the skin of patients with cystic fibrosis of the pancreas . Clin Chim Acta 36:113-117, 1972;.
9 +
Griffin GD, Gibbs GE:  Lysosomal enzymes of cultured white blood cells in cystic fibrosis . Proc Soc Exp Biol Med 137:438-442, 1971;.
10 +
Marchi AG, Mangiarotti Marchi MA, Nordio S:  Studio di attivita enzimatiche lisosomiali in linfociti fito-stimolati nella fibrosi cistica del pancreas . Minerva Pediatr 23:1842-1846, 1971;.
11 +
Antonowicz I, Sippell WG, Schwachman H:  Lysosomal and mitochondrial enzyme activities of lymphoid cell lines . Pediatr Res 6:803-812, 1972;.
12 +
Benke PJ:  Beta glucuronidase and cystic fibrosis . N Engl J Med 284:731-732, 1971;.
13 +
Knaus I, et al:  Metachromasia and assay for lysosomal enzymes in skin fibroblasts cultured from patients with cystic fibrosis and controls . Pediatrics 47:1010-1018, 1971;.
14 +
Butterworth J, et al:  Lysosomal enzymes of cultured fibroblasts of cystic fibrosis patients . Clin Chim Acta 40:139-142, 1972;.
15 +
Antonowicz I, Schwachman H:  Beta-galactosidase and beta-glucuronidase in intestinal mucosa . Pediatrics 47:737-744, 1971;.
16 +
Maxfield M, Wolins W:  A molecular abnormality of urinary mucoprotein in cystic fibrosis of the pancreas . J Clin Invest 41:455-462, 1972;.
17 +
Danes BS, Bearn AG:  Cystic fibrosis: Distribution of mucopolysaccharides in fibroblast cultures . Biochem Biophys Res Commun 36:919-924, 1969;.
18 +
Crosby P, Marsden JC, Robards AW:  Electron microscopy of urinary glycoproteins and its relation to cystic fibrosis . Histochem J 3:223-232, 1971;.

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