Live Cell FRET Microscopy
Open accessAbstract
The adrenoleukodystrophy protein (ALDP) and the 70-kDa peroxisomal membrane protein (PMP70) are half-ATP-binding cassette (ABC) transporters in the mammalian peroxisome membrane. Mutations in the gene encoding ALDP result in a devastating neurodegenerative disorder, X-linked adrenoleukodystrophy (X-ALD) that is associated with elevated levels of very long chain fatty acids because of impaired peroxisomal beta-oxidation. The interactions of peroxisomal ABC transporters, their role in the peroxisomal membrane, and their functions in disease pathogenesis are poorly understood. Studies on ABC transporters revealed that half-transporters have to dimerize to gain functionality. So far, conflicting observations are described for ALDP. By the use of in vitro methods (yeast two-hybrid and immunoprecipitation assays) on the one hand, it was shown that ALDP can form homodimers as well as heterodimers with PMP70 and ALDR, while on the other hand, it was demonstrated that ALDP and PMP70 exclusively homodimerize. To circumvent the problems of artificial interactions due to biochemical sample preparation in vitro, we investigated protein-protein interaction of ALDP in its physiological environment by FRET microscopy in intact living cells. The statistical relevance of FRET data was determined in two different ways using probability distribution shift analysis and Kolmogorov-Smirnov statistics. We demonstrate in vivo that ALDP and PMP70 form homodimers as well as ALDP/PMP70 heterodimers wher…
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Two different studies found the very C-terminal region of ABCD1 to play a major role in homodimerization and heterodimerization of ABCD1.
“However, two different studies found the very C-terminal region to play a major role in homodimerization and heterodimerization of ABCD1.81,82”
From The genetic landscape of X-linked adrenoleukodystrophy: inheritance, mutations, modifier genes, and diagnosis (2015) · cites this paper as [82]
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Peroxisomal ABC transporters: Structure, function and role in disease
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Structure-Function Analysis of Peroxisomal ATP-binding Cassette Transporters Using Chimeric Dimers
Geillon, F., Gondcaille, C., Charbonnier, S., Van Roermund, C. W., Lopez, T. E., Dias, A. M. M., Pais de Barros, J.-P., Arnould, C., Wanders, R. J., Trompier, D., et al. (2014) · Journal of Biological Chemistry · doi:10.1074/jbc.m114.575506 · Cited by 24 · see citing works
- Authors
- Hillebrand, M., Verrier, S. E., Ohlenbusch, A., Schäfer, A., Söling, H.-D., Wouters, F. S., & Gärtner, J.
- Affiliations
- University of Göttingen (DE)
- Max Planck Institute for Biophysical Chemistry (DE)
- European Neuroscience Institute Göttingen (DE)
- Corresponding author
- Hillebrand, Merle
- Gärtner, Jutta
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 2007-09
- Volume
- 282
- Issue
- 37
- Pages
- 26997-27005
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W2059824676
- Cited by
- 54
- References
- 4
- Citations by year
- 2026: 1 · 2025: 1 · 2023: 2 · 2022: 1 · 2021: 4 · 2020: 2 · 2019: 2 · 2018: 3 · 2017: 1 · 2016: 2 · 2015: 5 · 2014: 3 · 2013: 8 · 2012: 2
- FWCI
- 2.63
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Biological Chemistry (publishedVersion, cc-by)
- PubMed (publishedVersion)
- GRO (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Health Sciences › Medicine › Oncology › Drug Transport and Resistance Mechanisms
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Keywords
- PMP70, ABC transporters, peroxisomal membrane, protein-protein interaction, homodimerization, heterodimerization, X-linked adrenoleukodystrophy, very long chain fatty acids, peroxisomal beta-oxidation, C-terminal domain, transmembrane domain, FRET imaging, live-cell imaging
- MeSH terms
- ATP Binding Cassette Transporter, Subfamily D, Member 1, Adrenoleukodystrophy, Base Sequence, Humans, Microscopy, Molecular Sequence Data, Structure-Activity Relationship, ATP-Binding Cassette Transporters, Dimerization, Fluorescence Resonance Energy Transfer
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Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
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