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24 results on '"Yue Zou"'

22. General Mitochondrial Trifunctional Protein (TFP) Deficiency as a Result of Either a- or ß-Subunit Mutations Exhibits Similar Phenotypes Because Mutations in Either Subunit Alter TFP Complex Expression and Subunit Turnover

Author

Spiekerkoetter, Ute, Khuchua, Zaza, Yue, Zou, Bennett, Michael J, and Strauss, Arnold W

Abstract

The mitochondrial trifunctional protein (TFP) is a multienzyme complex of the ß-oxidation cycle. Human TFP is an octamer composed of four a-subunits harboring long-chain enoyl-CoA hydratase and long-chain L-3-hydroxyacyl-CoA dehydrogenase and four ß-subunits encoding long-chain 3-ketoacyl-CoA thiolase. Mutations in either subunit may result in general TFP deficiency with reduced activity of all three enzymes. We report five new patients with a-subunit mutations and compare general TFP deficiency caused by a-subunit mutations (n = 15) to that caused by ß-subunit mutations (n = 13) with regard to clinical features, enzyme activity, mutations, thiolase expression, and thiolase protein turnover. Among patients with a-subunit mutations, the same three heterogeneous phenotypes reported in patients with ß-subunit mutations were observed: a lethal form with predominating cardiomyopathy; an infancy-onset, hepatic presentation; and a milder, later-onset, neuromyopathic form. Maternal HELLP syndrome (hemolysis, elevated liver enzymes, low platelets) occurred with an incidence of 15 to 20%, as in families with ß-subunit mutations. Enzyme assays in fibroblasts revealed an identical biochemical pattern in both groups. a-Subunit mutational analysis demonstrated molecular heterogeneity, with 53% (9 of 17) truncating mutations. In contrast, patients with ß-subunit mutations had predominantly missense mutations. Thiolase expression in fibroblasts was as markedly reduced in a-subunit patients as in the ß-subunit group with similarly increased thiolase degradation, presumably secondary to TFP complex instability. TFP deficiency as a result of either a- or ß-subunit mutations presents with similar, heterogeneous phenotypes. Both a- and ß-subunit mutations result in TFP complex instability, demonstrating that the mechanism of disease is the same in a- or ß-mutation-derived disease and explaining the biochemical and clinical similarities.

Published
2004
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23. Fracture of Nanostructured Ionomer Membranes.

Author

Gdoutos, E. E., Yue Zou, Huang, X., and Reifsnider, K. L.

Abstract

Thin ionomer membranes showing nanostructure phase separation are widely used in low temperature fuel cells as electrolytes. Examples include perfluorinated sulfonic acid (PFSA) ionomers, better known as Nafion™ produced by E. I. du Pont de Nemours and Company. Their electrochemical performance and mechanical strength properties are determined by their molecular morphology, which resembles water-filled hydrophilic micellae (4∼5nm in size) dispersed in a hydrophobic matrix. Temperature and humidity are two major parameters in an internal fuel cell working environment, which have significant influence on this nanostructure and consequently influence the material properties. The transport properties of such ionomers have been extensively studied. In this paper, we will discuss fracture and strength of such membranes, as related to humidity and temperature. [ABSTRACT FROM AUTHOR]

Published
2006
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