Dutch home-based pre-reading intervention with children at familial risk of dyslexia

Children (5 and 6 years old, n = 30) at familial risk of dyslexia received a home-based intervention that focused on phoneme awareness and letter knowledge in the year prior to formal reading instruction. The children were compared to a no-training at-risk control group (n = 27), which was selected a year earlier. After training, we found a small effect on a composite score of phoneme awareness (d = 0.29) and a large effect on receptive letter knowledge (d = 0.88). In first grade, however, this did not result in beneficial effects for the experimental group in word reading and spelling. Results are compared to three former intervention studies in The Netherlands and comparable studies from Denmark and Australia

Diets containing sea cucumber (Isostichopus badionotus) meals are hypocholesterolemic in young rats

Peer reviewedPublisher PD

Role of ketone bodies in perinatal myocardial energy metabolism

Metabolic changes at around the time of birth are crucial for life. Here we review the energy utilization in the myocardium, emphasizing ketone body metabolism. Before birth, glucose and lactate are the major energy substrates for the myocardium. Long-chain fatty acids (LCFA) are normally not available as an energy substrate for the fetal heart; however, when LCFA are supplied artificially in near-term fetal lambs, they are readily oxidized. Hence the myocardium has no limitation to its ability to use LCFA before birth. After birth, lactate remains an important energy source for the myocardium, whereas the contribution of glucose to myocardial energy production decreases despite an increase in the supply of glucose. The oxidation of ketone bodies increases after birth in relation to an increase in supply. However, ketone bodies account for only 7% of left ventricular oxygen consumption. The supply and contribution of LCFA to the myocardium increases after birth; the oxidation of LCFA accounts for most of the left Ventricular oxygen consumption. Hence the role of ketone bodies in myocardial metabolism is limited. However, there are interesting observations on interference between the uptake of different substrates and the release of ketone bodies, which might have consequences for our interpretation of ketone body utilization

Rationale for a conditional knockout mouse model to study carnitine palmitoyltransferase I deficiencies

Several severe congenital cardiomyopathies are known to be associated with deficiencies in long-chain fatty acid transport and oxidation. Our studies are focused on a key enzyme in the regulation of intracellular long-chain fatty acid transport: carnitine palmitoyltransferase 1. Of this enzyme, two isoforms are expressed in the neonatal heart: L-CPT1 (the "liver-type" isoform) and M-CPT1 (the "muscle-type" isoform). It is known from studies in rats that chemical inhibition of both CPT1 isoforms results in hypertrophy of the cardiomyocytes, leading to an increase in heart-weight of up to 25%. With the aid of expressed sequence tag database analyses, cDNA- and genomic sequence information, we analysed the human gene for M-CPT1 in detail, and obtained partial clones of the murine genes for both CPT1 isoforms. We now started the development of a conditional knockout model to analyse and dissect deficiencies in these genes. While of the other mitochondrial components of the carnitine system deficiencies are known, some with severe cardiac consequences, M-CPT1 deficiencies have never been described. This suggests that M-CPT1 deficiency either(1) has not been recognised within the pool of congenital disorders, (2) is detrimental in an early stage of reproduction or embryogenesis, or (3) does not lead to physiological problems, probably due to the existence of a rescue system. If (1) is the case, the phenotypic effects of M-CPT1 deficiency have to be studied in order to generate criteria for clinical decision making and diagnosis. Option (2) demonstrates the necessity to use novel vector systems to create conditional gene disruptions. Hypothesis (3) implies a possible role for L-CPT1, and a knockout model allows a study of the interaction between the genes for L-CPT1 and M-CPT1. Applicable strategies to develop such a model system will be discussed.</p