pathology of skeletal muscle in mitochondrial disorders

... Gadolinium dependence of the ryanodine binding of the channel. Ryanodine binding in the presence of 18 n M [ 3 H]ryanodine were determined at various gadolinium concentrations. Solid line represents ... the visualization of the protein composition of the fractions. Ryanodine-binding Ryanodine-binding assays were carried out using [ 3 H]ry- anodine in a medium having the composition of 1 m NaCl, S. ... [4-(2-aminoethyl)-benzolsulfonyl- fluoride], 100 nm aprotinin, 1 lm leupeptin, 1 lm pepstatin A and 1 mm benzamidine. Protein concentration of HSR vesicles was determined according to the method of...

Ngày tải lên: 19/02/2014, 16:20

... 0.05; Fig. 5G). Discussion In this study, in an attempt to identify proteins involved in the physiopathology of muscle wasting, we examined the variation in the expression levels of sev- eral atrophy-associated ... family of transcription regulators known as muscle ankyrin repeat proteins. These three isoforms share in their C-terminal region a minimal structure composed of several ankryrin-like domains possibly ... mdx 4Cv muscles. Staining with dystrophin (in red) was used to delimit the fibres. A view of each muscle taken with a 40· objective is presented, showing the very specific staining observed within clusters...

Ngày tải lên: 07/03/2014, 03:20

... calcium-dependent inhibition of muscle contraction. TnC and TnI interact in an antiparallel fashion in which the N domain of TnC binds in a calcium-dependent manner to the C domain of TnI, releasing the inhibitory ... 117 and 148 are involved in the binding to N domain of TnC [11–16]. Serial deletion studies of the C terminus of TnI showed that residues 166–182 are involved in binding to the thin filament [16]. ... TnI region involved in the binding in the N domain of TnC. TnC can be cross-linked to residues 113–145 of TnI [11]. Pearlstone et al. [13] using a recombinant fragment of TnI con- taining residues...

Ngày tải lên: 16/03/2014, 18:20

... that of F-actin alone (dotted line in Fig. 10). As shown in F ig. 10, the behaviour of actin in the actin±HMM complex was quite different from that of F-actin alone, indicating that the actin±HMM ... intrinsic ¯uorescence of ATP-G-actin Following pressure increase, a red shift in the spectra with a decrease in the intrinsic ¯uorescence intensity of G-actin was observed (Fig. 1, inset). Fig. 1 shows ... that of eATP bo und to G-actin. Therefore, the increase of ¯uorescence seems to b e due mainly to the conformational change of actin under pressure. In situ pressure-induced changes in the intensity of...

Ngày tải lên: 24/03/2014, 00:21

... staining of P-glycoprotein Cytospins of GLC4 and GLC4 ⁄ P-glycoprotein cells were incubated for 1 min with hematoxylin for staining of nuclei. To assess the localization of P-glycoprotein expres- sion ... neither mitochondria, inhibition of calcineurin nor blockage of P-glycoprotein play a role in the comparable induction of ROS production by CsA and its analogs. We found that CsA induces a strong increase in JC-1 fluorescence ... accumulates in cells as a con- sequence of P-glycoprotein inhibition, resulting in an apparent increase in DW m . We also subsequently tested whether P-glycoprotein pumps are involved in the accu- mulation...

Ngày tải lên: 30/03/2014, 08:20

Ngày tải lên: 20/06/2014, 00:20

... immunoblotting data, it was of interest to re-examine the fate of cals equestrin by studying the entire complement of key Ca 2+ -binding elements in dystrophin- deficient skeletal muscle fibres. ... software. Results In order to determine the fate of the terminal cisternae Ca 2+ -binding protein, calsequestrin, and related luminal sarcoplasmic reticulum elements i n dystrophin-deficient skeletal muscle, ... racking dye] t o achieve a fi nal protein concentration o f 50 lg of p rotein per IEF strip for silver staining, hot Coomassie s taining, ÔStains-AllÕ labelling or immunoblotting. T he following...

Ngày tải lên: 19/02/2014, 16:20

... was developed in the hind leg of mice by inject- ing 3MC, a chemical carcinogen, or by injecting into the hind leg existing sarcoma 180 cells, which had been previously maintained in the peritoneal ... protein applied. MCK sMitC K β-actin NM ST Fig. 5. Expression of mRNA of MCK and sMitCK isoforms of crea- tine kinase and b-actin in normal muscle (NM) and 3MC induced sarcoma tissue (ST) of mouse. 1.0 0.8 0.6 0.4 0.2 Relative ... concerning the levels of creatine and CK in malignant cells and in tumor-bearing animals. In some recent publications, the ratio of choline to crea- tine has been measured in several types of brain tumors...

Ngày tải lên: 07/03/2014, 06:20

... binding to titin by improving the accessibility of the titin-binding domain. Interestingly, the importance of CARP inter- action for its function was recently demonstrated in a Fig. 3. Calpain ... calpain 3 was investigated. A direct, in vitro digestion of CARP by calpain 3 using recombinant proteins could not be attempted because calpain 3 is inactivated during purifi- cation [45]. In skeletal ... major inducer of muscle atrophy in the skeletal muscle. Indeed, NF-jB inhibi- tion in mice models invariably protects against muscle atrophy, whereas NF-jB activation promotes proteoly- sis in vivo...

Ngày tải lên: 29/03/2014, 21:20

Ngày tải lên: 18/06/2014, 16:20

Ngày tải lên: 21/06/2014, 02:20

... characterized by insulin resistance, and thus the inability of insulin to stimulate glucose utilization in skeletal muscle. It has been proposed that insulin resistant individuals have a defect in GLUT4 ... that expressed in skeletal muscle. Our results using human skeletal muscle suggest that hSGLT3 might be involved in glucose transport following progressive resistance training in diabetic patients. ... patients. Based on our findings, the co-localization of hSGLT3 with the nicotinic acetylcholine receptor in skeletal muscle at baseline prior to any exercise training, is in accordance with other...

Ngày tải lên: 31/10/2012, 17:08

... [26]. Identification of mitochondrial import sequences in iPLA 2 c and demonstration of their functional integrity Recent increases in the understand ing of mitochondrial import machinery have led ... virtue of the absence of any N-terminal mitochondrial leader sequence in this isoform. Discussion After t he initial discovery of a novel intrace llular c alcium- independent PLA 2 activity in myocardium ... Studies of insulin secretory responses and of arachidonic acid incorporation into phospholipids of stably transfected insulinoma cells that overexpress group VIA phos- pholipase A 2 (iPLA2beta) indicate...

Ngày tải lên: 19/02/2014, 16:20

... role of recombination in mtDNA inheritance in S. cerevisiae showed that budding cells were enriched in linear monomers of mtDNA. It was proposed that in addition to a role in initiating a rolling- circle ... recombination could be instrumen- tal in resolving concatamers into linear monomers in the process of mtDNA partitioning and transmission into buds [11]. Several groups identified the Ydc2 protein of ... lacking leucine in the presence of thiamine for inhibition of the nmt promoter. For visualization of Ydc2–GFP constructs, yeast cultures were grown in selective media in the absence of thiamine...

Ngày tải lên: 20/02/2014, 11:20

... 7B and C). Effects of this deletion on growth of yeast cells were similar in strains containing mitochondrial group II introns and in strains lacking these introns. Accordingly, the ARM deletion ... amino acids in the central part of Mrs2 proteins (Fig. 1). We defined five sequence blocks containing various charged amino acid residues (CRB-1–5). Three of them (CRB-3–5) are in the vicinity of ... coiled-coil domains and adjacent conserved sequences either are involved in oligomerization of the Mrs2p channel protein or in forming structures participating in the gating of this channel....

Ngày tải lên: 23/03/2014, 10:21

... deficiency in argininosuccinate synthetase (ASS). In the liver, the AGC plays an important role in the urea cycle by providing aspartate for incorporation into argininosuccinate [17]. The mutations in ... Katsunuma, T. (1980) Role of argininosuccinate synthetase in the regulation of urea synthesis in the rat and argininosuccinate synthetase-asso- ciated metabolic disorders in man. Adv. Enzyme Regul. ... respectively). (D) Immunoblotting of increasing amounts of recombinant citrin and aralar1. Known amounts (as indicated) of bacterially expressed aralar1 and citrin N-terminal regions were loaded on...

Ngày tải lên: 24/03/2014, 04:21

... this differ- ence is of doubtful importance in clinical practise. Thus, the inclu- sion of BW as an independent predictor of TBSMM will only slightly increase the degree of explanation, and it ... FFM. There is an increasing interest to specifically estimate skeletal muscle mass (SMM), as it may better reflect the body protein reserves and nutritional status in disease and aging. 4 SMM loss (sarcopenia) ... Study of Women in Go ¨ te- borg and contributed to study design and writing of the manu- script, Ingvar Bosaeus initiated and designed the present study and contributed to analysing data and writing...

Ngày tải lên: 28/03/2014, 20:20

... AMP-activated protein kinase c3, has a key role in glucose and fat metabolism in skeletal muscle, as suggested by metabolic characterization of humans, pigs and mice harboring substitutions in the AMP-binding ... AMP-activated protein kinase kinase; CAMKKb,Ca 2+ ⁄ calmodulin-dependent protein kinase b; CBS, cystathionine-b-synthase; TAK1, transforming growth factor b-activating kinase 1; ZMP, 5-aminoimidazole-4-carboxamide ... of c1-containing AMPK trimers, when recombinant upstream kinase and AMPK preparations were used [44,47]. In line with these studies, we did not detect any increase in phos- phorylation of a2ina2b2c3...

Ngày tải lên: 30/03/2014, 08:20