Abstract:
:Homozygous calsequestrin 2 (CASQ2) point mutations leads to catecholaminergic polymorphic ventricular tachycardia: a common pathogenetic feature appears to be the drastic reduction of mutant CASQ2 in spite of normal transcription. Comparative biochemical analysis of R33Q and D307H knock in mutant mice identifies different pathogenetic mechanisms for CASQ2 degradation and different molecular adaptive mechanisms. In particular, each CASQ2 point mutation evokes specific adaptive cellular and molecular processes in each of the four adaptive pathways investigated. Thus, similar clinical phenotypes and identical cellular mechanism for cardiac arrhythmia might imply different molecular adaptive mechanisms.
journal_name
J Muscle Res Cell Motiljournal_title
Journal of muscle research and cell motilityauthors
Valle G,Arad M,Volpe Pdoi
10.1007/s10974-020-09587-2subject
Has Abstractpub_date
2020-09-01 00:00:00pages
251-258issue
2-3eissn
0142-4319issn
1573-2657pii
10.1007/s10974-020-09587-2journal_volume
41pub_type
杂志文章abstract::The time course of shortening at zero load was studied by the slack test method during tetanic contractions in isolated, single, slow muscle fibres of the crayfish. In 28 of 32 shortenings (from 14 different fibres) a biphasic shortening was seen, which consisted of an initial high-velocity phase lasting 3.3-20.8 ms a...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF01738251
更新日期:1992-12-01 00:00:00
abstract::We investigated the expression of myosin subunits (myosin heavy chains) as well as light chains and the in vivo phosphorylation of the phosphorylatable myosin light chain in the heart ventricle of the adult male European hamster (Cricetus cricetus L.). Two myosin heavy chain isoenzymes could be detected under native a...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF01738429
更新日期:1992-02-01 00:00:00
abstract::Several factors have an influence on the improvement of muscle activity and motor co-ordination of mammals during post-natal development. One of them is voltage sensitive L-type calcium channel function. In striated muscles of adult mammals these channels are located in T-tubule membranes thus linking the on-coming ac...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1010305421661
更新日期:2001-01-01 00:00:00
abstract::Results were obtained from contracting frog muscles by collecting high quality time-resolved, two-dimensional, X-ray diffraction patterns at the British Synchrotron Radiation Source (SERC, Daresbury, Laboratory). The structural transitions associated with isometric tension generation were recorded under conditions in ...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00123096
更新日期:1993-06-01 00:00:00
abstract::The remodelling of connective tissue components is a fundamental requirement for a number of pivotal processes in cell biology. These may include myoblast migration and fusion during development and regeneration. In other systems, similar biological processes are facilitated by secretion of the matrix metalloproteinas...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1005670507906
更新日期:2000-04-01 00:00:00
abstract::We have investigated high-affinity ryanodine-binding sites in membrane preparations from representative fast-twitch and slow-twitch muscles of the rabbit and rat, as well as from human mixed muscle. Our results, obtained in high-ionic strength binding buffer, demonstrate extensive similarities in binding affinity for ...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00130421
更新日期:1994-04-01 00:00:00
abstract::The effects of a ramp stretch (amplitude <6% muscle fibre length (L0), speed < 13L0 s(-1)) on twitch tension and twitch tension re-development were examined in intact mammalian (rat) fast and slow muscle fibre bundles. The experiments were done in vitro at 20 degrees C and at an initial sarcomere length of 2.68 microm...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1010556623905
更新日期:2001-01-01 00:00:00
abstract::In isolated myosin the reaction sequence of essential thiol groups with N-ethylmaleimide was studied using the following five approaches: kinetics of the modification reaction, effects of modification on enzyme properties, affinity chromatography of isolated subfragment-1 stemming from modified myosin, isolation of cy...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00711923
更新日期:1980-03-01 00:00:00
abstract::In frog semitendinosus muscle the descending limb of the length-tension curve is shifted rightward relative to that of tibialis anterior. Both the plateau right corner and the zero-force intercept are equally shifted. To investigate the reason for this shift, we compared filament lengths in the two muscles. Single fib...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00115451
更新日期:1993-04-01 00:00:00
abstract::Nebulin is an integral protein of skeletal muscle thin filaments and probably acts as a ruler for the thin filament length. Cardiac muscles of higher vertebrates have been shown earlier to lack nebulin. Instead in human and chicken cardiac muscle the much smaller protein nebulette replaces nebulin. Since nebulette is ...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1020909902462
更新日期:2002-01-01 00:00:00
abstract::Myosin binding protein C (MYBPC) is a crucial component of the sarcomere and an important regulator of muscle function. While mutations in different myosin binding protein C (MYBPC) genes are well known causes of various human diseases, such as hypertrophic (HCM) and dilated (DCM) forms of cardiomyopathy as well as sk...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章,评审
doi:10.1007/s10974-011-9281-6
更新日期:2012-05-01 00:00:00
abstract::Rotary-shadowed filamin molecules appear as long, highly flexible rods curved into a variety of configurations. The particles observed were 2.7 nm wide but had contour lengths of either 98 nm or 193 nm. The longer particles are probably end-to-end dimers of the shorter but it is not clear how many polypeptide chains t...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00711869
更新日期:1981-06-01 00:00:00
abstract::Cytokinesis is the mechanical process that allows the simplest unit of life, the cell, to divide, propagating itself. To divide, the cell converts chemical energy into mechanical energy to produce force. This process is thought to be active, due in large part to the mechanochemistry of the myosin-II ATPase. The cell's...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章,评审
doi:10.1023/a:1024419510314
更新日期:2002-01-01 00:00:00
abstract::The characteristics of the titin filament in the vicinity of the Z-line were investigated using immunoelectron microscopy. We used monoclonal titin antibodies T-11 and T-12 on single fibres of frog skeletal muscle, and on Z-line-extracted fibres. It is well established that the I-band region of titin is elastic. We fi...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00121293
更新日期:1993-08-01 00:00:00
abstract::We describe the modification of reactive actin sulfhydryls by S-nitrosoglutathione. Kinetics of S-nitrosylation and denitrosylation suggest that only one cysteine of actin is involved in the reactions. By using the bifunctional sulfhydryl cross-linking reagent N,N'-1,4-phenylenebismaleimide and the monofunctional reag...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1005671319604
更新日期:2000-02-01 00:00:00
abstract::The cellular function of the giant protein titin in striated muscle is a major focus of scientific attention. Particularly, its role in passive mechanics has been extensively investigated. In strong contrast, the structural details of this filament are very poorly understood. To date, only a handful of atomic models f...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-005-9017-6
更新日期:2005-01-01 00:00:00
abstract::The aim of the study was to investigate sex-related differences in contractile properties, parameters of action potentials, and mechanisms of force regulation of motor units in the rat soleus muscle, which is a frequent experimental model in animal research. It was revealed that the mean mass of the muscle in males wa...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-015-9408-2
更新日期:2015-06-01 00:00:00
abstract::Long-(SL > 6 microns) and short-sarcomere (SL < 4 microns) fibres were isolated from the claw muscle of the yabby (Cherax destructor) during limb regeneration and at different stages of the moult cycle. Long-sarcomere fibres were more susceptible to the changes resulting from the moult-induced atrophy compared with th...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00121136
更新日期:1995-06-01 00:00:00
abstract::In mammalian ventricles, two myosin heavy chain (MHC) isoforms have been identified. Small animals express α-MHC, whereas large animals express β-MHC, which contribute to a large difference in the heart rate. Sprague-Dawley rats possessing ~99% α-MHC were treated with propylthiouracil to result in 100% β-MHC. Papillar...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-016-9456-2
更新日期:2016-12-01 00:00:00
abstract::The change in orientation of myosin crossbridges in contracting muscle during sudden length changes was examined by fluorescence polarization. This study used a fluorescent ATP analogue, 1,N6-etheno-2-aza-ATP(epsilon-2-aza-ATP) as a probe. Its fluorescence is considerably enhanced upon binding with myosin and is depen...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00712310
更新日期:1985-02-01 00:00:00
abstract::We investigated the regulatory roles of MgADP and free Ca2+ in isometric tension development in skinned bovine cardiac muscle. We found that, in the relaxed state without free Ca2+, MgADP elicited a sigmoidal increase in active tension, as is the case in skeletal muscle (ADP-contraction). The critical MgADP concentrat...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1005437517287
更新日期:1998-11-01 00:00:00
abstract::A balanced redox status is necessary to optimize force production in contractile apparatus, where free radicals generated by skeletal muscle are involved in some basic physiological processes like excitation-contraction coupling. Protein glutathionylation has a key role in redox regulation of proteins and signal trans...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-008-9145-x
更新日期:2008-01-01 00:00:00
abstract::High speed isovelocity shortening using a servo-controlled lever was performed on isolated whole frog sartorius muscles at long lengths to ensure substantial passive tension. The tension records of unstimulated control experiments were subtracted from the tension records of fully-tetanized experiments on the same musc...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00123833
更新日期:1994-02-01 00:00:00
abstract::Troponin T (TnT) is one of the muscle regulatory proteins and is thought to be related to unique contractile properties in diverse muscles and also to myogenesis. The expression of TnT isoforms in the chicken wing muscles was investigated by two-dimensional gel electrophoresis and immunoblotting with an antiserum agai...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1016369300532
更新日期:2001-01-01 00:00:00
abstract::The non-hydrolysable ATP analogue, beta,gamma-imido-adenosine-5'-triphosphate (AMPPNP, adenylilimidodiphosphate) does not reduce the stiffness of demembranated sea urchin sperm flagella measured in the rigor state in the absence of MgATP2- and does not cause relaxation of rigor bends. The competitive inhibitions provi...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/BF00712064
更新日期:1981-03-01 00:00:00
abstract::Following activation by G-protein-coupled receptor agonists, protein kinase C (PKC) modulates cardiac myocyte function by phosphorylation of intracellular targets including myofilament proteins cardiac troponin I (cTnI) and cardiac myosin binding protein C (cMyBP-C). Since PKC phosphorylation has been shown to decreas...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-012-9294-9
更新日期:2012-12-01 00:00:00
abstract::Under muscle disuse conditions decrease of expression of MyHC of slow type, and sometimes of type IIa, as well as upregulation of expression of IIb and IId/x isoforms were observed. Through dephosphorylation and entry of NFAT molecules to the nucleus calcineurin/NFATc1 signaling pathway promotes upregulation of the sl...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-015-9428-y
更新日期:2016-04-01 00:00:00
abstract::The term 'microtubule dynamics' is often used to describe assembly/disassembly characteristics of this important cytoskeletal polymer. The ability to image microtubules in live Dictyostelium cells has revealed additional dynamic components, acting on the individual assembled tubules. At least two separate forces are i...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章,评审
doi:10.1023/a:1024446821701
更新日期:2002-01-01 00:00:00
abstract::In Drosophila melanogaster two high molecular weight tropomyosin isoforms, historically named heavy troponins (TnH-33 and TnH-34), are encoded by the Tm1 tropomyosin gene. They are specifically expressed in the indirect flight muscles (IFM). Their N-termini are conventional and complete tropomyosin sequences, but thei...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1007/s10974-005-9044-3
更新日期:2006-01-01 00:00:00
abstract::Guinea-pig soleus muscles were denervated and electrically stimulated for periods of 43 to 66 days. Stimuli were in 1 s bursts of 40 Hz pulses, repeated every 5 min. Other guinea-pigs were denervated for 82 days without stimulation and, in a third group, the soleus muscle was necrotized and allowed to regenerate witho...
journal_title:Journal of muscle research and cell motility
pub_type: 杂志文章
doi:10.1023/a:1018619503456
更新日期:1997-10-01 00:00:00