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Originally published In Press as doi:10.1074/jbc.C300093200 on March 6, 2003

J. Biol. Chem., Vol. 278, Issue 18, 16310-16314, May 2, 2003
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A Glutamine to Proline Exchange at Amino Acid Residue 1098 in Sucrase Causes a Temperature-sensitive Arrest of Sucrase-isomaltase in the Endoplasmic Reticulum and cis-Golgi*

Marcus J. Pröpsting, Ralf Jacob, and Hassan Y. NaimDagger

From the Department of Physiological Chemistry, School of Veterinary Medicine, D-30559 Hannover, Germany

A striking feature of phenotype II in congenital sucrase-isomaltase deficiency is the retention of the brush border protein sucrase-isomaltase (SI) in the cis-Golgi. This transport block is the consequence of a glutamine to proline substitution at amino acid residue 1098 of the sucrase subunit. Here we provide unequivocal biochemical and confocal data to show that the SIQ/P mutant reveals characteristics of a temperature-sensitive mutant. Thus, correct folding, competent intracellular transport, and full enzymatic activity can be partially restored by expression of the mutant SIQ/P at the permissive temperature of 20 °C instead of 37 °C. The acquisition of normal trafficking and function appears to utilize several cycles of anterograde and retrograde steps between the endoplasmic reticulum and the Golgi implicating the molecular chaperones calnexin and heavy chain-binding protein. The data presented in this communication are to our knowledge the first to implicate a temperature-sensitive mutation in an intestinal enzyme deficiency or an intestinal disorder.

* This work was supported by Deutsche Forschungsgemeinschaft Grant Na 331/1-2 (to H. Y. N.) and Sonderforschungsbereich Grant 621 (to H. Y. N. and R. J.).The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Dagger To whom correspondence should be addressed: Dept. of Physiological Chemistry, School of Veterinary Medicine Hannover, Bünteweg 17, D-30559 Hannover, Germany. Tel.: 49-511-9538780; Fax: 49-511-9538585; E-mail: Hassan.Naim@tiho-hannover.de.

Copyright © 2003 by The American Society for Biochemistry and Molecular Biology, Inc.
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