Adrenoleukodystrophy; Alzheimer Disease; ATP-Binding Cassette Transporters; Evolution, Molecular; Lipid Metabolism; Peroxisomes; Protein Transport
Intracellular Transport Processes
A variety of metabolic pathways are sequestered within peroxisomes. Thus, the participating enzymes and the metabolic substrates have to be imported into this organelle. The mechanisms mediating an efficient and specific transport of soluble and membrane proteins to peroxisomes are a major focus of interest. The transfer of small organic compounds across the peroxisomal membrane by specific transporter proteins and the metabolic interconversion of these compounds within peroxisomes are the second major focus. Both aspects are of medical importance, because a dysfunction in transport processes has been linked to human diseases such as X-linked adrenoleukodystrophy, Alzheimers disease or metabolic disorders.
Techniques, methods & infrastructure
Immunofluorescenc Microscopy, Western Blot, Subcellular Fractionation, Density Gradient Centrifugation, Yeast Two Hybrid Assay, Mammalian Two Hybrid Assay, Immunoprecipitation, Cell Culture Techniques, PCR, qRT-PCR
- Berger, J. et al., 2016. Peroxisomes in brain development and function. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 1863(5), pp.934-955. Available at: http://dx.doi.org/10.1016/j.bbamcr.2015.12.005.
- Kunze, M. & Berger, J., 2015. The similarity between N-terminal targeting signals for protein import into different organelles and its evolutionary relevance. Frontiers in Physiology, 6. Available at: http://dx.doi.org/10.3389/fphys.2015.00259.
- Kunze, M. et al., 2014. Mechanistic Insights into PTS2-mediated Peroxisomal Protein Import. Journal of Biological Chemistry, 290(8), pp.4928-4940. Available at: http://dx.doi.org/10.1074/jbc.M114.601575.
- Hoftberger R*/Kunze M* (*joint authorship), Voigtlander T, Unterberger U, Regelsberger G, Bauer J, Aboul-Enein F, Garzuly F, Forss-Petter S, Bernheimer H, Berger J, Budka H (2010) Peroxisomal localization of the proopiomelanocortin-derived peptides Ss-Lipotropin and Ss-Endorphin Endocrinology, 151: 4801-4810
- Wiesinger, C. et al., 2013. Impaired Very Long-chain Acyl-CoA β-Oxidation in Human X-linked Adrenoleukodystrophy Fibroblasts Is a Direct Consequence of ABCD1 Transporter Dysfunction. Journal of Biological Chemistry, 288(26), pp.19269-19279. Available at: http://dx.doi.org/10.1074/jbc.M112.445445.