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Wilson, William C.,Hornig-Do, Hue-Tran,Bruni, Francesco,Chang, Jeong Ho,Jourdain, Alexis A.,Martinou, Jean-Claude,Falkenberg, Maria,Spå,hr, Henrik,Larsson, Nils-Gö,ran,Lewis, Richard J.,Hewit Oxford University Press 2014 Human Molecular Genetics Vol.23 No.23
<P>The p.N478D missense mutation in human mitochondrial poly(A) polymerase (mtPAP) has previously been implicated in a form of spastic ataxia with optic atrophy. In this study, we have investigated fibroblast cell lines established from family members. The homozygous mutation resulted in the loss of polyadenylation of all mitochondrial transcripts assessed; however, oligoadenylation was retained. Interestingly, this had differential effects on transcript stability that were dependent on the particular species of transcript. These changes were accompanied by a severe loss of oxidative phosphorylation complexes I and IV, and perturbation of <I>de novo</I> mitochondrial protein synthesis. Decreases in transcript polyadenylation and in respiratory chain complexes were effectively rescued by overexpression of wild-type mtPAP. Both mutated and wild-type mtPAP localized to the mitochondrial RNA-processing granules thereby eliminating mislocalization as a cause of defective polyadenylation. <I>In vitro</I> polyadenylation assays revealed severely compromised activity by the mutated protein, which generated only short oligo(A) extensions on RNA substrates, irrespective of RNA secondary structure. The addition of LRPPRC/SLIRP, a mitochondrial RNA-binding complex, enhanced activity of the wild-type mtPAP resulting in increased overall tail length. The LRPPRC/SLIRP effect although present was less marked with mutated mtPAP, independent of RNA secondary structure. We conclude that (i) the polymerase activity of mtPAP can be modulated by the presence of LRPPRC/SLIRP, (ii) N478D mtPAP mutation decreases polymerase activity and (iii) the alteration in poly(A) length is sufficient to cause dysregulation of post-transcriptional expression and the pathogenic lack of respiratory chain complexes.</P>
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HOW ACCURATE ARE COMMERCIAL BRAND VALUATION METHODS?
Marc Fischer,Rex Du,Tobias Hornig 글로벌지식마케팅경영학회 2018 Global Marketing Conference Vol.2018 No.07
Many commercial vendors provide brand valuation measures, which have important practical implications for many stakeholders on how brands are bought and sold and how they are managed. These commercial measures, however, differ to a great extent from one another, which raises serious concerns about their accuracy. In this study, we assess the measurement properties of brand valuation measures provided by the four dominant commercial vendors: Interbrand, Brand Finance, Millward Brown, and Corebrand. We find that the brand valuation measures from the four commercial vendors measure the same underlying construct in a reliable and valid way according to established psychometric test methodology. However, it appears that they measure a construct that does not predict actual brand transaction prices well. In most cases, they significantly overestimate the transaction price. The magnitude of systematic bias varies by method and industry. Our results suggest that an upward bias is more likely to occur in situations when the relevance of tangible assets and non-brand intangible assets is high. These empirical findings challenge current practical and academic thinking on brand valuation. A main conclusion is that brand valuation for transaction purposes needs to first value all assets of the company in their totality and then allocate a fair share to the brand.
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