نتایج جستجو برای: myofibrillar myopathy
تعداد نتایج: 14255 فیلتر نتایج به سال:
Myofibrillar protein catabolic rate was calculated in seven patients with Duchenne muscular dystrophy from the amount of 3-methylhistidine excreted in the urine, and found to be over three times that found in a control series when expresses as the percentage of myofibrillar protein catabolised per day. It is suggested that measurement of myofibrillar protein catabolic rate may add a useful para...
hereditary inclusion body myopathy (hibm) is an adult-onset hereditary myopathy, usually with distal onset and quadriceps sparing. this myopathy is autosomal recessive and associated to upd-n-acetylglucosamine-2-epimerase/n-acetylmannosamine kinase (gne) gene mutations. in this study, we report a novel gne homozygous point mutation c.1834t>g that results in amino acid substitution of cysteine 6...
We examined the effects of serum deprivation on myofibrillar proteolysis in chick myotubes. Myotubes were incubated with serum-free medium for 24 hours. N(tau)-methylhistidine release, as an index of myofibrillar proteolysis, as well as protease activities such as calpain, proteasome, and cathepsins (B+L and D) activities were increased by serum deprivation. These results indicate that serum de...
Six-day-old chick skeletal muscle (extensor digitorum longus) was incubated in the presence of corticosterone (CTC; 0, 3, 30, and 300 ng/ml) for 2 h at 37 degrees C. Tyrosine and N (tau)-methylhistidine releases, as indices of total muscle and myofibrillar proteolysis, were increased by CTC but with different dose responses, indicating an independent regulation of myofibrillar and non-myofibril...
A cardioskeletal myopathy with onset and death in infancy, morphological features of muscle type I hypotrophy with myofibrillar disorganization and dilated cardiomyopathy was previously reported in three Dutch families. Here we report the genetic cause of this disorder. Multipoint parametric linkage analysis of six Dutch patients identified a homozygous region of 2.1 Mb on chromosome 12, which ...
ABSTRACT To determine the usefulness of a method of percutaneous needle muscle biopsy in patients with suspected idiopathic inflammatory Myopathy. The yield of percutaneous needle muscle biopsy was studied in 15 patients during 1997-2000 who were hospitalized in Hazrat Rasool Akram hospital. A diagnosis of idiopathic inflammatory myopathy was confirmed histopathologically in 8 patients...
We studied the effect of protein and protein-free energy intake on the degradation rate of myofibrillar protein in preruminant calves in two experiments. Both experiments were similar in design but were performed with two live weight ranges: 80 to 160 kg (Exp. 1) and 160 to 240 kg (Exp. 2). In each experiment, calves were allocated to either an initial slaughter group or to one of 12 treatments...
Knocking out myostatin activity during development increases the rate of muscle protein synthesis. The present study was done to determine whether postdevelopmental loss of myostatin activity stimulates myofibrillar protein synthesis and the phosphorylation of some of the proteins involved in regulation of protein synthesis rate. Myostatin activity was inhibited for 4 days, in 4- to 5-mo-old ma...
1. The urinary excretion of 3-methylhistidine and creatinine was measured in 15 controls and in two groups of 15 patients with liver cirrhosis, with and without severe muscle wasting. All subjects were on a meat-free diet. The values obtained were used to calculate the fractional catabolic rate of myofibrillar protein. 2. In patients without muscle wasting 3-methylhistidine excretion was high i...
نمودار تعداد نتایج جستجو در هر سال
با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید