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Third-party-funded project

Title:
Molecular and mechanistic investigation of alpha-methylacyl-CoA racemase

Project management at the University of Würzburg:

Participating scientists:

Abstract:
The enzyme alpha-methylacyl-CoA racemase is localized mainly in peroxisomes (in humans). It is required for the degradation of cholesterol to bile acids and for the ß-oxidation of branched-chain fatty acids. In the present research project, we intend - in continuation of our previous studies - to work on the following topics:
1. Analysis of the targeting signals and of the different organelle distribution in rat, mouse and humans.
2. Expression of larger amounts of the enzymatically active protein from various species in suitable systems, crystallization and X-ray structure analysis.
3. Biochemical analysis of a transgenic racemase-deficient mouse model. (Collaboration with Prof. K. Hiltunen, University of Oulu, Finland.)
4. Molecular analysis of fibroblasts from patients with deficiency of the enzyme.

Key words:
    Lipid Storage Diseases
    Cholesterol Degradation
    Alpha-methylacyl-CoA racemase
    Protein targeting
    Branched chain fatty acids

Projekt period: from 04.1998 to 09.2002

Funding institution:
DFG ,Granting date: 01.04.1998

Publications: