BACKGROUND In a recent study, we showed that administration of low-dose creatine (Cr) (60 mg/kg daily) improved work capacity in patients with McArdle disease. OBJECTIVE To assess the efficacy of high-dose Cr therapy in McArdle disease. DESIGN Randomized, double-blind, placebo-controlled crossover study. PATIENTS Nineteen patients with McArdle disease. INTERVENTION Treatment with Cr, 15...

32 McArdle disease (glycogen storage disease type V) is caused by inherited deficiency of 33 a key enzyme in muscle metabolism, the skeletal-muscle specific isoform of glycogen 34 phosphorylase, ‘myophosphorylase’, which is encoded by the PYGM gene. Here we 35 review the main pathophysiological, genotypic and phenotypic features of McArdle 36 disease and their interactions. To date, moderate-in...

The present study examined the relative contributions of the different pathways by which oxidatively modified VLDL (oxVLDL) promotes the uptake and intracellular accumulation of lipids in J774 macrophages. VLDL was oxidized for a maximum of 4 hours, resulting in an increase in thiobarbituric acid-reactive substances and an increased electrophoretic mobility on agarose gel. The lipid composition...

Oxidative modification of low density lipoprotein (LDL) generates a form that is degraded much more rapidly by macrophages and may thus be more atherogenic than unoxidized LDL. Recently, we provided evidence that oxidative modification of LDL may play a significant role in the generation of fatty streaks in the LDL receptor-deficient rabbit. The major lipoprotein in cholesterol-fed animals is t...

Previous studies from our laboratory (J Upld Res 1988;29:643-656) have shown that thioglycolate-eliclted peritoneal macrophages from White Carneau and Show Racer pigeons, like mammalian macrophages, have on their surfaces specific receptors for acetyiated low density llpoproteln (acLDL) and ^-migrating very low density lipoprotelns (0-VLDL). The binding kinetics of 0-VLDL were complex, however,...

Watanabe Heritable Hyperlipidemic (WHHL) rabbits are an important animal model used to study the effects of defective low density lipoprotein (LDL) receptors on lipoprotein metabolism. In the present study, the receptor-mediated catabolism of apolipoprotein (apo) E-containing lipoproteins was investigated in fibroblasts and smooth muscle cells cultured from WHHL rabbits. Fibroblasts from WHHL r...

The nonproliferating chicken liver cell culture system described yields cell monolayers with morphological and lipogenic properties characteristic of the physiological-nutritional state of donor animals. Synthesis and secretion of fatty acid, cholesterol, and very low density lipoprotein (VLDL) occur at in vivo rates and respond to hormones and agents which affect these processes in vivo. Cells...

Previous studies from our laboratory (J Lipid Res 1988;29:643-656) have shown that thioglycolate-elicited peritoneal macrophages from White Carneau and Show Racer pigeons, like mammalian macrophages, have on their surfaces specific receptors for acetylated low density lipoprotein (acLDL) and beta-migrating very low density lipoproteins (beta-VLDL). The binding kinetics of beta-VLDL were complex...

Previous studies from our laboratory (J Upld Res 1988;29:643-656) have shown that thioglycolate-eliclted peritoneal macrophages from White Carneau and Show Racer pigeons, like mammalian macrophages, have on their surfaces specific receptors for acetyiated low density llpoproteln (acLDL) and ^-migrating very low density lipoprotelns (0-VLDL). The binding kinetics of 0-VLDL were complex, however,...