Molecular aspects of disaccharidase deficiencies

Baillière's Clinical Gastroenterology
E E SterchiHassan Y Naim

Abstract

We have described the methods used for studying the biosynthesis and the post-translational processing of sucrase-isomaltase (SI), lactase-phlorizin hydrolase (LPH) and maltase-glucoamylase (MGA) in human small intestinal mucosa. Our results are discussed in the context of findings by other researchers. A surprising finding coming out of all these studies is that SI, LPH and MGA are structurally quite different. SI and LPH are both synthesized as large molecular weight precursors which are proteolytically processed to the mature enzymes. In the case of SI, this processing occurs after insertion of the precursor into the brush border membrane and is catalysed by pancreatic proteases; the mature form consists of the two subunits sucrase and isomaltase, the latter containing an N-terminal peptide anchor. Proteolytic processing of the LPH-precursor occurs intracellularly, yielding a mature enzyme in the form of a two active site polypeptide which is anchored via a C-terminal peptide. The role of the large cleaved propolypeptide of LPH is not yet known. MGA is the largest of the three disaccharidases, having a molecular weight of greater than 300 kDa. No proteolytic processing seems to be taking place during biogenesis of MGA in hum...Continue Reading

References

Feb 1, 1979·The Biochemical Journal·P R Flanagan, G G Forstner
Dec 1, 1978·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·J RothL Orci
Apr 1, 1976·The New England Journal of Medicine·G M GrayR R Townley
Dec 1, 1985·Journal of Pediatric Gastroenterology and Nutrition·H SjöströmE M Danielsen
Jan 14, 1986·Biochemical and Biophysical Research Communications·D H AlpersA W Strauss
Aug 1, 1986·Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire·L Lee, G Forstner
Jan 1, 1987·Advances in Human Genetics·G Flatz
Sep 1, 1988·Scandinavian Journal of Gastroenterology·E Gudmand-HøyerP R Madsen
Dec 1, 1985·Journal of Pediatric Gastroenterology and Nutrition·J E Riby, N Kretchmer
Jul 1, 1985·Proceedings of the National Academy of Sciences of the United States of America·H P HauriM J Lentze
Sep 6, 1974·Biochimica Et Biophysica Acta·H PreiserJ J Cerda
Dec 19, 1973·Biochimica Et Biophysica Acta·V ColomboG Semenza
Feb 28, 1972·Biochimica Et Biophysica Acta·S Schlegel-HaueterG Semenza
Oct 1, 1972·Biochemical Medicine·H L GreeneR H Herman
Jan 1, 1968·Analytical Biochemistry·A Dahlqvist
May 1, 1966·Acta paediatrica Scandinavica·K LaunialaJ K Visakorpi
Jan 1, 1982·Physiological Reviews·A J Kenny, S Maroux
Oct 1, 1980·Scientific American·C Milstein
Jan 1, 1984·Canadian Journal of Biochemistry and Cell Biology = Revue Canadienne De Biochimie Et Biologie Cellulaire·L Lee, G Forstner
Jul 18, 1984·Biochemical and Biophysical Research Communications·E M DanielsenH Sjöström
Sep 1, 1982·European Journal of Biochemistry·S H SørensenE M Danielsen
Apr 1, 1983·Archives of Disease in Childhood·E SavilahtiP Kuitunen
Mar 14, 1980·Clinica Chimica Acta; International Journal of Clinical Chemistry·E E Sterchi, J F Woodley
Jan 1, 1980·Journal of Immunological Methods·S F de StGroth, D Scheidegger
May 30, 1959·Lancet·A HOLZELK W SUTCLIFFE
Aug 6, 1960·Lancet·H A WEIJERSW K DICKE
Aug 13, 1962·Biochimica Et Biophysica Acta·R G DOELL, N KRETCHMER
Mar 1, 1965·The Journal of Pediatrics·S AURICCHIOM DAVIDSON

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