A KCNJ10 mutation previously identified in the Russell group of terriers also occurs in Smooth-Haired Fox Terriers with hereditary ataxia and in related breeds

Background

Hereditary ataxias with similar phenotypes were reported in the Smooth-Haired Fox Terrier, the Jack Russell Terrier and the Parson Russell Terrier. However, segregation analyses showed differing inheritance modes in these breeds. Recently, molecular genetic studies on the Russell group of terriers found independent mutations in KCNJ10 and CAPN1, each associated with a specific clinical subtype of inherited ataxia. The aim of this study was to clarify whether or not Smooth-Haired Fox Terriers with hereditary ataxia and dogs of other related breeds harbor either of the same mutations. A sub goal was to update the results of KCNJ10 genotyping in Russell group terriers.

Findings

Three Smooth-Haired Fox Terriers with hereditary ataxia and two Toy Fox Terriers with a similar phenotype were all homozygous for the KCNJ10 mutation. The same mutation was also found in a heterozygous state in clinically unaffected Tenterfield Terriers (n = 5) and, in agreement with previous studies, in Jack Russell Terriers, Parson Russell Terriers, and Russell Terriers.

Conclusions

A KCNJ10 mutation, previously associated with an autosomal recessive spinocerebellar ataxia in Jack Russell Terriers, Parson Russell Terriers, and Russell Terriers segregates in at least three more breeds descended from British hunting terriers. Ataxic members of two of these breeds, the Smooth-Haired Fox Terrier and the Toy Fox Terrier, were homozygous for the mutation, strengthening the likelihood that this genetic defect is indeed the causative mutation for the disease known as “hereditary ataxia” in Fox Terriers and “spinocerebellar ataxia with myokymia, seizures or both” in the Russell group of terriers.     

An objective examination of consumer perception of nutrition information based on healthiness ratings and eye movements

OBJECTIVE: Previous research on nutrition labelling has mainly used subjective measures. This study examines the effectiveness of two types of nutrition label using two objective measures: eye movements and healthiness ratings. DESIGN: Eye movements were recorded while participants made healthiness ratings for two types of nutrition label: standard and standard plus the Food Standards Agency's 'traffic light' concept. SETTING: University of Derby, UK. SUBJECTS: A total of 92 participants (mean age 31.5 years) were paid for their participation. None of the participants worked in the areas of food or nutrition. RESULTS: For the standard nutrition label, participant eye movements lacked focus and their healthiness ratings lacked accuracy. The traffic light system helped to guide the attention of the consumer to the important nutrients and improved the accuracy of the healthiness ratings of nutrition labels. CONCLUSIONS: Consumers have a lack of knowledge regarding how to interpret nutrition information for standard labels. The traffic light concept helps to ameliorate this problem by indicating important nutrients to which to pay attention.     

Effect of type of oil and addition of delta-tocopherol on model flavor compound stability during storage

The objective of this study was to investigate approaches to protect selected flavor compounds from deterioration when stored in an oil matrix. An aroma compound model mixture was prepared in a medium-chain triglyceride (MCT) or sunflower oil (SfO) matrix and stored under either an ambient air or argon atmosphere containing, respectively, ca. 20 and <0.5% residual oxygen as controls or containing a natural antioxidant, delta-tocopherol (0.01%). Samples were analyzed by static headspace GC/FID to determine the stability over time of the compounds in mixture. It was found that the type of oil had the greatest effect (P < 0.01) on overall compound stability. A low-oxygen atmosphere also had a significant (P < 0.05) protective effect on the aroma compounds in both oils. The addition of delta-tocopherol generally offered little additional protection. No significant relationship could be determined between the oxidation of the lipid matrix and the loss of oxidation-sensitive thiol compounds.     

Synthesis and characterization of canola oil-stearic acid-based trans-free structured lipids for possible margarine application

Incorporation of stearic acid into canola oil to produce trans-free structured lipid (SL) as a healthy alternative to partially hydrogenated fats for margarine formulation was investigated. Response surface methodology was used to study the effects of lipozyme RM IM from Rhizomucor miehei and Candida rugosa lipase isoform 1 (LIP1) and two acyl donors, stearic acid and ethyl stearate, on the incorporation. Lipozyme RM IM and ethyl stearate gave the best result. Gram quantities of SLs were synthesized using lipozyme RM IM, and the products were compared to SL made by chemical catalysis and fat from commercial margarines. After short-path distillation, the products were characterized by GC and RPHPLC-MS to obtain fatty acid and triacylglycerol profiles, 13C NMR spectrometry for regiospecific analysis, X-ray diffraction for crystal forms, and DSC for melting profile. Stearic acid was incorporated into canola oil, mainly at the sn-1,3 positions, for the lipase reaction, and no new trans fatty acids formed. Most SL products did not have adequate solid fat content or beta' crystal forms for tub margarine, although these may be suitable for light margarine formulation.     

A preliminary study on the effects of dietary supplementation of brewers yeast and nucleotides, singularly or in combination, on juvenile red drum (Sciaenops ocellatus)

This study was designed to evaluate the effects of dietary supplementation with brewers yeast (Brewtech^®) and nucleotides (Optimûn), either singularly or in combination, on red drum growth, body composition, stress responses and possible resistance to Amyloodinium ocellatum infection. Juvenile red drum (～1 g initially) fed practical fish‐meal‐based diets for 6 weeks had similar weight gain regardless of whether the diet was unsupplemented (basal) or supplemented with brewers yeast (2% of diet), nucleotides (Optimûn at 0.2% of diet) or both brewers yeast (2% of diet) and nucleotides (Optimûn at 0.2% of diet). Dietary effects on hepatosomatic index, intraperitoneal fat ratio or whole‐body composition were not significant, except that fish fed the singular nucleotide supplement had a significantly higher whole‐body lipid content compared with fish fed the basal diet. No significant dietary effects on cortisol response were observed after a 15 min confinement stress test perhaps because of the extreme variation among individual fish. Subsequently, in situ challenge by co‐habitation with A. ocellatum caused 100% mortality regardless of dietary treatment within a 48 h period. It is concluded that dietary supplementation of brewers yeast and nucleotides did not promote enhanced growth or improved cortisol response and resistance to uncontrolled amyloodiniosis in juvenile red drum, at least at the tested dosages.