ACAN should be considered as a candidate gene in patients with short stature and minor skeletal defects, particularly those with brachydactyly, and in patients with spondyloepiphyseal dysplasia. It is also important to note that advanced BA and osteoarticular complications are not obligatory conditions for aggrecanopathies/aggrecan-associated dysplasias.

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The last two decades have witnessed an increasing enthusiasm of consumers for products with specific characteristics, whether related to origin or production technology. Several studies show that consumers are willing to pay price premium for organic products and we may expect that the same happens with other attributes, such as origin and tradition. The aim of this study, based on surveys conducted in the context of a taste panel, is to establish the price premium that consumers are willing to pay for the feature «traditional variety», using Portuguese traditional varieties of apples as a case study. Using contingent valuation to determine the willingness to pay and panel data techniques for estimation, a hedonic price model is developed to explain how the price that consumers declare to be willing to pay is related, besides variety, to different attributes of apples, such as appearance and taste, production method and threat of extinction. The results suggest that consumers, regardless of their socio-economic characteristics, are willing to pay a higher price not only for apples with better taste, appearance and smell, but also for Portuguese traditional varieties.

No abstract