It has been suggested that a gene termed bubblegum (Bgm), encoding an acyl-CoA synthetase, could be involved in the pathogenesis of the inherited neurodegenerative disorder X-ALD (X-linked adrenoleukodystrophy). The precise function of the ALDP (ALD protein) still remains unclear. Aldp deficiency in mammals and Bgm deficiency in Drosophila led to accumulation of VLCFAs (very long-chain fatty acids). As a first step towards studying this interaction in wild-type versus Aldp-deficient mice, we analysed the expression pattern of the murine orthologue of the Bgm gene. In contrast with the ubiquitously expressed Ald gene, Bgm expression is restricted to the tissues that are affected by X-ALD such as brain, testis and adrenals. During mouse brain development, Bgm mRNA was first detected by Northern-blot analysis on embryonic day 18 and increased steadily towards adulthood, whereas the highest level of Ald mRNA was found on embryonic day 12 and decreased gradually during differentiation. Protein fractionation and confocal laser imaging of Bgm–green fluorescent protein fusion proteins revealed a microsomal localization that was different from peroxisomes (where Aldp is detected), endoplasmic reticulum and Golgi. Mouse Bgm showed acyl-CoA synthetase activity towards a VLCFA substrate in addition to LCFAs, and this activity was enriched in the microsomal compartment. Speculating that Bgm expression could be regulated by Ald deficiency, we compared the abundance of Bgm mRNA in wild-type and Ald knockout mice but observed no difference. Although mouse Bgm is capable of activating VLCFA, we conclude that a direct interaction between the mouse Bgm and the Aldp seems unlikely.
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January 2004
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Research Article|
January 01 2004
Murine bubblegum orthologue is a microsomal very long-chain acyl-CoA synthetase
Peter FRAISL;
Peter FRAISL
Division of Neuroimmunology, Brain Research Institute, Vienna University Medical School, Spitalgasse 4, 1090 Vienna, Austria
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Sonja FORSS-PETTER;
Sonja FORSS-PETTER
Division of Neuroimmunology, Brain Research Institute, Vienna University Medical School, Spitalgasse 4, 1090 Vienna, Austria
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Mihaela ZIGMAN;
Mihaela ZIGMAN
Division of Neuroimmunology, Brain Research Institute, Vienna University Medical School, Spitalgasse 4, 1090 Vienna, Austria
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Johannes BERGER
Johannes BERGER
1
Division of Neuroimmunology, Brain Research Institute, Vienna University Medical School, Spitalgasse 4, 1090 Vienna, Austria
1To whom correspondence should be addressed (e-mail johannes.berger@univie.ac.at).
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Publisher: Portland Press Ltd
Received:
July 16 2003
Revision Received:
September 26 2003
Accepted:
September 30 2003
Accepted Manuscript online:
September 30 2003
Online ISSN: 1470-8728
Print ISSN: 0264-6021
The Biochemical Society, London ©2004
2004
Biochem J (2004) 377 (1): 85–93.
Article history
Received:
July 16 2003
Revision Received:
September 26 2003
Accepted:
September 30 2003
Accepted Manuscript online:
September 30 2003
Citation
Peter FRAISL, Sonja FORSS-PETTER, Mihaela ZIGMAN, Johannes BERGER; Murine bubblegum orthologue is a microsomal very long-chain acyl-CoA synthetase. Biochem J 1 January 2004; 377 (1): 85–93. doi: https://doi.org/10.1042/bj20031062
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