Primary amniotic fluid cell, skin fibroblast and liver alpha-L-fucosidase and its relation to cystic fibrosis

Clinica Chimica Acta; International Journal of Clinical Chemistry
J Butterworth, G J Guy

Abstract

Cultured skin fibroblast and primary amniotic fluid cell alpha-L-fucosidase had a double optimum of pH 5.0 and 6.0. Alpha-L-fucosidase was largely bound as a single peak to DEAE-cellulose at pH 6.6. Sucrose density isoelectric focusing revealed up to seven components with pI values of 4.9, 5.2, 5.4, 5.8, 6.1, 6.5 and 7.1 with their apparent KM values (77--500 mumol/l) being higher than that (57 mumol/l) of the unfocused enzyme. Liver, skin fibroblast and amniotic fluid cell alpha-L-fucosidase was separated into two peaks by gel filtration. Peak one was more active and stable at low pH and more thermostable at 50 degrees C than peak two, while both peaks had an apparent KM of 52 mumol/l. Apart from the different proportions of the peaks separated by gel filtration, the results for the three tissues were similar. The properties of alpha-L-fucosidase studied were similar for control and cystic fibrosis liver or skin fibroblasts.

References

Dec 2, 1977·European Journal of Clinical Pharmacology·B Beermann, M Groschinsky-Grind
Dec 7, 1977·Biochemical and Biophysical Research Communications·T F ScanlinM C Glick
Jun 1, 1975·FEBS Letters·R Thorpe, D Robinson
Dec 1, 1975·The Journal of Pediatrics·N G BeratisK Hirschhorn
Jun 1, 1968·Lancet·F Van Hoof, H G Hers
Jul 1, 1972·The Journal of Experimental Medicine·K ZielkeJ S O'Brien

Citations

Oct 26, 1981·Clinica Chimica Acta; International Journal of Clinical Chemistry·A Harris, D Swallow
Jun 16, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·J Butterworth, D Priestman
Aug 18, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·E M Beyer, G Y Wiederschain
Jan 5, 1982·Clinica Chimica Acta; International Journal of Clinical Chemistry·A F Bury, R C Roberts
Apr 25, 1983·Clinica Chimica Acta; International Journal of Clinical Chemistry·C BourassaM Potier

Related Concepts

Amniotic Fluid Index
Chromatography, DEAE-Cellulose
Molecular Sieve Chromatography
Pulmonary Cystic Fibrosis
Fucosidase
Hydrogen-Ion Concentration
Isoelectric Focusing
Liver
Skin

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