Characterization and kinetics studies of water buffalo (Bubalus bubalis) myoglobin

The colour of buffalo (Bubalus bubalis L.) meat is darker than bovine meat. Since meat colour depends on the concentration of myoglobin (Mb) and its oxidation state, we have determined the main structural and functional properties of buffalo Mb. Buffalo Mb was purified from longissimus dorsi muscles and its molecular mass determined by ESI Q-TOF mass spectrometry. The molecular mass 17,034.50 was 86.20 Da higher than the bovine Mb. This was confirmed by analysing its primary structure, using a combined approach based on Edman degradation and MALDI-TOF mass spectrometry. Comparing the amino acid sequences of both Mbs, we found three amino acid differences out of 153 amino acid residues. One is a conservative substitution (Dbov141Ebuf), and the other two (Abov19Tbuf and Abov117Dbuf) are nonconservative. These amino acid substitutions are unlikely to cause structural changes because they are located far from the heme binding pocket, as revealed by the 3D structure of buffalo Mb elaborated by homology modelling. Stability analyses show no difference with the bovine Mb for helix E and only minor differences in the stability values for helices A and G. Moreover, autoxidation rates of purified buffalo and bovine myoglobins at 37 °C, pH 7.2, were almost identical, 0.052 ± 0.001 h- 1 and 0.054 ± 0.002 h- 1, respectively, as were their oxygen-binding Kd values, 3.7 ± 0.1 μM and 3.5 ± 0.1 μM, respectively. The percent of MetMb values were almost identical. The results presented here suggest that the darker buffalo meat depends on factors other than the oxidation rate of its Mb, as, for example, the Mb content (0.393 ± 0.005 g/100 g of tissue) and consequently MetMb, which are almost twice as high as bovine meat (Mb: 0.209 ± 0.003 g/100 g of tissue). © 2006 Elsevier Inc. All rights reserved

Kinetics of amyloid aggregation of mammal apomyoglobins and correlation with their amino acid sequences

AbstractIn protein deposition disorders, a normally soluble protein is deposited as insoluble aggregates, referred to as amyloid. The intrinsic effects of specific mutations on the rates of protein aggregation and amyloid formation of unfolded polypeptide chains can be correlated with changes in hydrophobicity, propensity to convert α-helical to β sheet conformation and charge. In this paper, we report the aggregation rates of buffalo, horse and bovine apomyoglobins. The experimental values were compared with the theoretical ones evaluated considering the amino acid differences among the sequences. Our results show that the mutations which play critical roles in the rate-determining step of apomyoglobin aggregation are those located within the N-terminal region of the molecule

Expanding the clinical and genetic heterogeneity of SPAX5

Mutations in the ATPase family 3-like gene (AFG3L2) have been linked to autosomal-dominant spinocerebellar ataxia type 28 and autosomal recessive spastic ataxia-neuropathy syndrome. Here, we describe the case of a child carrying bi-allelic mutations in AFG3L2 and presenting with ictal paroxysmal episodes associated with neuroimaging suggestive of basal ganglia involvement. Studies in skin fibroblasts showed a significant reduction of AFG3L2 expression. The relatively mild clinical presentation and the benign course, in spite of severe neuroimaging features, distinguish this case from data reported in the literature, and therefore expand the spectrum of neurological and neuroradiological features associated with AFG3L2 mutations

Demand Dynamics With Socially Evolving Preferences

this paper witnessed at a seminar the horror of most colleagues when Werner Hildebrand, presenting some further development on his theory of demand (Hildebrand (1994)) provocatively suggested more or less that "preferences and choices are matters for psychiatrists and not for economists", while the task of the latter should be primarily to establish some statistical conditions under which basic propositions of economic theory -- such as downward sloping demand curves etc. -- hold in the aggregate, in presence of heterogeneous, and possibly "irrational" consumers. In a nutshell, the provocation highlights, first of all, a major divide cutting across the economic discipline -- as well as other social sciences --, namely, how seriously should one take standard utility theory (with or without its more recent refinements) and the associated "rational" theory of decision making as the foundation of a descriptive theory of demand? (Another major problem concerns the aggregate properties of diverse demand schedules, no matter how constructed: we shall come to that below.) Needless to say, the majority of the economic profession seems to take that type of microeconomic foundations of decisions very seriously indeed, entrenched as they are with deep ("anthropological") views on the nature of "rationality" and self-seeking behaviours, passed through successive generations via conventional teaching tools such as "indi#erence curves" and the like, and further justified by their purported role in bridging descriptive and normative analyses (welfare theorems, etc.). However, admittedly minority views in economics (but nearly "mainstream" in other social disciplines) claim that classic decision theory has little to o#er by way of the interpretation of what people actually do and that o..

Developmental Coordination Disorder Questionnaire (DCDQ): traduzione e adattamento alla lingua italiana e studio pilota su bambini con DCD e DSA.

Il DCDQ (BN Wilson, 2000) è uno strumento largamente utilizzato per valutare le competenze motorie tra i 4 e i 14 anni. Abbiamo effettuato l’adattamento cross-culturale seguendo le linee guida correnti. Il DCDQ è stato compilato dai genitori di 26 bambini con DCD, 52 bambini con sviluppo tipico appaiati per età e genere. I punteggi dei singoli items e del totale sono risultati statisticamente differenti tra i due gruppi (p<.01). Le analisi delle proprietà psicometriche mostrano ottimi valori di consistenza interna, specificità/sensibilità e affidabilità del questionario. Abbiamo poi condotto uno studio pilota su bambini con DSA (N=22) confrontando i punteggi del DCDQ con quelli di 16 bambini con DCD e 18 controlli. I risultati indicano una differenza significativa (p<.01) tra i gruppi sia nel punteggio totale che nelle singole aree ‘Controllo motorio’, ‘Motricità fine/grafia’ e ‘Coordinazione generale’. I bambini con DSA differiscono significativamente dai controlli esclusivamente nell’area di motricità fine/grafia

Nutritional and metabolic profiling of the globe artichoke (Cynara scolymus L. &apos;Capuanella&apos; heads) in province of Caserta, Italy

Abstract Globe artichoke (Cynara scolymus L.) is a typical vegetable of the countries of the Mediterranean basin. It produces edible immature flower bud and his cultivation has a great economic importance in Italy, first world producer. In the recent years, local cultivars have been gradually replaced with the modern ones, mostly due to the increasing demand of early products for the large-scale retail trade. The valorization of local resources, such as specific cultivars useful in productive processes, may contrast this tendency. In this framework, the aim of this research is the nutritional and metabolic characterization of the Cynara scolymus L. &apos;Capuanella&apos;, a typical artichoke of the Campania Region. The nutritional profile (i.e. moisture content, dietary fiber, ash, total proteins, lipids and carbohydrates, total and free amino acids, fatty acid composition, folic acid, C vitamin, total phenolic compounds) of edible immature flower bud of globe artichoke &apos;Capuanella&apos; was determined and compared to the Italian artichoke nutritional profile reported in the National Institute of Research on Food and Nutrition (INRAN) tables. On a fresh weight basis, the Capuanella artichoke total protein content was higher in comparison with the INRAN tables (3.08 vs. 2.70g/100 g, respectively), whereas essential amino acids were lower in Capuanella with respect to the INRAN values (826.3 vs. 884 mg/100 g, respectively). Among the fatty acids of Capuanella artichoke, the most abundant were the essential n-6 linoleic (55.20 mg/100 g) and palmitic (34.80 mg/g) acids, representing about 72% of the total fatty acids. Ascorbic acid was 13.70 mg/100 g, while folic acid represents 17% of Recommended Dietary Allowance (65.00 µg/100 g). Phenolic compounds were found to be abundant in Capuanella artichoke (1878.21 mg/100 g); in particular, the chlorogenic acid (425.46 mg/100 g) represents about 23% of total phenolic compounds. Finally, as confirmed by the AFLP analysis, the Capuanella artichoke belongs to the &quot;Romanesco&quot; group

Free amino acid profile in Malus domestica cv Annurca apples from the Campania region and other Italian vegetables

The apple Malus domestica Borkh cv Annurca is a variety from the Campania Region (Southern Italy) known for its high quality and recognized with the ”Protected Geographical Indication” (PGI) trademark. Differences in cultivation area could help to guarantee the geographic origin of this product. Free amino acids of Annurca apples cultivated in the Campania Region were determined on an amino acid analyzer with post-column ninhydrin derivatization procedure and utilized in a chemometric approach. The main free amino acids present in samples from Annurca apples were asparagine (>70 mg/100 g), aspartic acid, glutamic acid and ophosphoserine (about 1-5 mg/100 g each). Other amino acids were detected in minor amounts (less than 1 mg/100 g). Subsequently, the cluster analysis indicated that the Annurca apples from Presenzano, Pietravairano, Vitulazio and Valle di Maddaloni (little villages of the Campania Region) may be distinguished on the basis of their free amino acid profiles. The confidence of the cluster obtained for apples, was tested considering the free amino acid profiles of other Italian vegetables. It is concluded that free amino acid profiles are good indicators of the geographic origin of Annurca apples and other vegetables, proposing a simple method compared with more tedious ones, which could be transferred to other laboratories, making easier the acquisition of raw data (free amino acid profiles)