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 skeletal muscular disorders, the underlying molecular mechanisms remain not well understood. A variety of MYBPC3 (cardiac isoform) mutations have been studied in great detail and several corresponding genetically altered mouse models have been generated. Most MYBPC3 mutations may cause haploinsufficiency and with it they may cause a primary increase in calcium sensitivity which is potentially able to explain major features observed in HCM patients such as the hypercontractile phenotype and the well known secondary effects such as myofibrillar disarray, fibrosis, myocardial hypertrophy and remodelling including arrhythmogenesis. However the presence of poison peptides in some cases cannot be fully excluded and most probably other mechanisms are also at play. Here we shall discuss MYBPC interacting proteins and possible pathways linked to cardiomyopathy and heart failure.
journal_name
J Muscle Res Cell Motiljournal_title
Journal of muscle research and cell motilityauthors
Knöll Rdoi
10.1007/s10974-011-9281-6subject
Has Abstractpub_date
2012-05-01 00:00:00pages
31-42issue
1eissn
0142-4319issn
1573-2657journal_volume
33pub_type
杂志文章,评审abstract：:Although a substantial literature exists on the in vitro polymerization of purified myosin, little is known about native thick filament assembly, remodeling or turnover. We have recently described a cell-free system (Bouche et al., 1988) to examine the interactions between thick filaments and soluble, newly synthesize...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF01774035
更新日期：1991-04-01 00:00:00
abstract：:In this study the spontaneous activities of prescapular lymph nodes, which were isolated from calves, goats and sheep and mesenteric lymph nodes of guinea-pigs were recorded and analysed in the frequency domain. Stretch-evoked contractions of the mesenteric lymph nodes were also recorded and their frequency characteri...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF00711961
更新日期：1983-02-01 00:00:00
abstract：:Striated muscle fine structure began to be really understood following a comprehensive survey of the matter carried out by William Bowman in the late 1830s. The publications resulting from such a study, the first of which earned for the author a precocious election as Fellow of the Royal Society, are herewith examined...
journal_title：Journal of muscle research and cell motility
pub_type： 传,历史文章,杂志文章
doi：10.1007/s10974-005-9048-z
更新日期：2006-01-01 00:00:00
abstract：:Heart disease is a leading cause of death in patients with Duchenne muscular dystrophy (DMD). Patients with DMD lack the protein dystrophin, which is widely expressed in striated muscle. In skeletal muscle, the loss of dystrophin results in dramatically decreased expression of the dystrophin associated glycoprotein co...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/s10974-013-9362-9
更新日期：2013-12-01 00:00:00
abstract：:In this study alterations are characterized which occur, in myocardial force development morphological appearance and protein composition, during the development of cardiac hypertrophy and heart failure in monocrotaline (MCT) treated rats. The transition from cardiac hypertrophy to heart failure was studied by compari...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1019988815436
更新日期：2002-01-01 00:00:00
abstract：:While the migration of capillary endothelial cells is believed central to the process of new blood vessel development in vivo, the biochemical basis for endothelial motility is unknown. Herein, we demonstrate that retina-derived growth factor (RDGF), a mitogen for endothelial cells (EC), stimulates the migration of mi...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF00713928
更新日期：1984-12-01 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：:The in vivo state of assembly of myosin in vertebrate smooth muscle is controversial. In vitro studies on purified smooth muscle myosin show that it is monomeric (10S) under relaxing conditions and filamentous under contraction conditions. Electron microscopic and antibody labelling studies of intact smooth muscles, o...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1018634412117
更新日期：1997-06-01 00:00:00
abstract：:Calcium overload is a fundamental pathogenic event associated with chronic muscle degeneration in muscular dystrophies. The possibility that L-type voltage-dependent calcium channels were involved in the etiology of chicken muscular dystrophy was investigated by studying the dihydropyridine receptors in transverse tub...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF00126437
更新日期：1995-10-01 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：:Several mutations in the beta-myosin heavy chain gene cause hypertrophic cardiomyopathy. This study investigates (1) the in vitro velocities of translocation of fluorescently-labelled actin by beta-myosin purified from soleus muscle of 30 hypertrophic cardiomyopathy patients with seven distinct beta-myosin heavy chain...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1018613907574
更新日期：1997-06-01 00:00:00
abstract：:Isolated frog muscle fibres of four different lengths (L0, initial; L1, 15%; L2, 25%; and L3, 35% stretched vs. L0) were stimulated continuously (3 min) by suprathreshold rectangular pulses at three different frequencies (f1 = 5 Hz, f2 = 6.7 Hz and f3 = 10 Hz). Using a pair of electrodes mounted at a fixed distance, t...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：
更新日期：1998-01-01 00:00:00
abstract：:Isolated fibres from various muscles were skinned mechanically in oil. From a Ca2+-loaded micropipette, local applications of Ca2+ were made. These produced a limited contraction which relaxed spontaneously. The time-course of sarcomere shortening and re-lengthening was recorded by microcinephotography. Application of...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1018603032590
更新日期：1997-08-01 00:00:00
abstract：:The trimeric protein AMP-activated protein kinase (AMPK) is an important sensor of energetic status and cellular stress, and mutations in genes encoding two of the regulatory γ subunits cause inherited disorders of either cardiac or skeletal muscle. AMPKγ2 mutations cause hypertrophic cardiomyopathy with glycogen depo...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/s10974-013-9359-4
更新日期：2013-12-01 00:00:00
abstract：:Quantitative (muscle fibre number and cross-sectional areas) and qualitative (myosin isoforms and metabolic enzyme activities) characteristics of two muscles, M. pectoralis major and M. anterior latissimus dorsi, were compared among male chickens of two lines during growth from hatching to adulthood. The lines were de...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF00122527
更新日期：1995-04-01 00:00:00
abstract：:To study the development of muscle-specific features during myogenesis, we analysed the ultrastructure and voltage-dependent currents of frog embryonic skeletal myocytes maintained in culture for 10 days. The cells were maintained under culture conditions that prevented cell division, fusion and cell contacts with neu...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1005506216009
更新日期：1999-08-01 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 length dependency of the sensitivity to activators of the smooth muscle of different blood vessels is not yet fully understood. Muscle preparations of the aorta, the femoral artery and the portal vein of the rabbit were investigated for the length dependency of the sensitivity to phenylephrine and calcium in both ...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1005408015885
更新日期：1999-01-01 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：:Gaps in our understanding of muscle mechanics demonstrate that the current model is incomplete. Increasingly, it appears that a role for titin in active muscle contraction might help to fill these gaps. While such a role for titin is increasingly accepted, the underlying molecular mechanisms remain unclear. The goals ...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/s10974-019-09540-y
更新日期：2020-03-01 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：: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
abstract：:Skeletal muscle hypertrophy is a result of increased load, such as functional and stretch-overload. Activation of satellite cells and proliferation, differentiation and fusion are required for hypertrophy of overloaded skeletal muscles. On the contrary, a dramatic loss of skeletal muscle mass determines atrophy settin...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章,评审
doi：10.1007/s10974-010-9204-y
更新日期：2009-12-01 00:00:00
abstract：:Clenbuterol is a beta2-adrenoceptor agonist primarily used for treating bronchospasm and alleviating the symptoms of chronic obstructive pulmonary disease (COPD) in the horse. In other species (rats, mice, sheep, and cattle), chronic high doses of clenbuterol (typically in the milligram per kilogram body weight range)...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1023/a:1027377731137
更新日期：2003-01-01 00:00:00
abstract：:Secophalloidin (SPH) is known to cause in cardiac myofibrils force without Ca(2+) (half-maximal effect approximately 2 mM) followed by irreversible loss of Ca(2+)-activated force. At maximal Ca(2+) activation, SPH increases force (half-maximal effect < 0.1 mM). We found that SPH at low concentration (0.5 mM) did not c...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/s10974-009-9188-7
更新日期：2009-01-01 00:00:00
abstract：:Critical illness myopathy (CIM) is associated with severe muscle atrophy and fatigue in affected patients. Apoptotic signaling is involved in atrophy and is elevated in muscles from patients with CIM. In this study we investigated underlying mechanisms of apoptosis-related pathways in muscles with different fiber type...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/s10974-015-9410-8
更新日期：2015-06-01 00:00:00
abstract：:Creatine kinase (CK) isoenzyme compositions of different types of single muscle fibres dissected from soleus (SOL) and extensor digitorum longus (EDL) muscles from rats were examined. CK isoenzymes were separated into cytoplasmic (CK-MM, CK-MB, CK-BB) (muscle, brain and hybrid types, respectively) and mitochondrial (m...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF01781172
更新日期：1991-02-01 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：:Myosin heavy chain kinase activity was identified in the high speed supernate of lysed Dictyostelium amoebae and was precipitated by 30-50% ammonium sulphate. In low ionic strength buffer, the activity bound tightly to a Cibacron Blue Sepharose column and eluted as a single peak with 1.0 M NaCl. Gel filtration chromat...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章
doi：10.1007/BF01753566
更新日期：1986-12-01 00:00:00
abstract：:Reactive oxygen species (ROS) are generated in skeletal muscle both during the rest and contractile activity. Myogenic cells are equipped with antioxidant enzymes, like superoxide dismutase, catalase, glutathione peroxidase, γ-glutamylcysteine synthetase and heme oxygenase-1. These enzymes not only neutralise excessiv...
journal_title：Journal of muscle research and cell motility
pub_type： 杂志文章,评审
doi：10.1007/s10974-015-9438-9
更新日期：2015-12-01 00:00:00
