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Dystrophin-Associated Proteins (2):
Recent article
Dystrophin-Associated Proteins
Laboratory Investigation (90)11 2010
Abstract
We investigated the involvement of dystrophin-associated proteins (DAPs) and their relationship with the perivascular basement membrane in the brains of mdx mice and controls at the age of 2 months. We analyzed (1) the expression of glial DAPs α-β-dystroglycan (DG), α-syntrophin, aquaporin-4 (AQP...
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PMID: 20714324
PDF is available here.
PDF is available here.
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Experimental Cell Research (316)14 2010
Abstract
We performed immunohistochemical analyses with a specific antibody to Syn2 and demonstrated predominant expression in the larval and adult central nervous system. To investigate the in vivo functions of Syn2, we have generated Drosophila Syn2 deficiency mutants. Although the Syn2 mutants exhibit no...
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PMID: 20632467
PDF is available here.
PDF is available here.
Neuroreport (21)10 2010
Abstract
We have shown that localization of the scaffolding proteins gamma2-syntrophin, alpha-dystrobrevin-2, and dystrophin to glial endfeet is also dependent on the presence of alpha-syntrophin. In this study, we show that the expression levels of alpha-syntrophin, gamma2-syntrophin, and dystrophin at the...
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PMID: 20508543
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
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PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
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PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
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PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
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PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
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PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
|
PMID: 19784870
PDF is available here.
PDF is available here.
Neurochemical Research (35)3 2010
Abstract
We have adopted the PC12 cell line as in vitro cell model for studying Dp71 function in neuronal cells. These cells express a cytoplasmic (Dp71f) and a nuclear (Dp71d) isoform of Dp71 as well as various dystrophin-associated proteins (DAPs). In this study, we revealed by confocal microscopy analysis...
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PMID: 19784870
PDF is available here.
PDF is available here.
Clinical Neuropathology (29)4 2010
Abstract
Immunolabeling of dystrobrevin could be a useful marker in the diagnostic of neuromuscular diseases....
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PMID: 20569677
PDF is available here.
PDF is available here.
Abstract
Present study demonstrates a novel localization of beta-dystroglycan in rat brain. Beside the meningeal surface and the cerebral vessels where beta-dystroglycan immunopositivity has been described, now immunopositive solid bodies were found at the basis of ependymocytes. Since they proved to be roun...
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PMID: 18985737
PDF is available here.
PDF is available here.
Abstract
The dystrophin/dystrobrevin/dystrotelin superfamily is marked by a common constellation of domains whose juxtaposition is tightly constrained in all three subfamilies. These domains comprise a cluster of four closely packed EF hands, a ZZ domain, and two coiled-coil regions. In addition, the dystrop...
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PMID: 19392646
PDF is available here.
PDF is available here.
Circulation Journal (73)1 2009
Abstract
Left ventricular noncompaction (LVNC) is a recently defined cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses, and is thought to be caused by arrest of normal endomyocardial morphogenesis. Although LVNC has been classified as a primary cardi...
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PMID: 19057090
PDF is available here.
PDF is available here.
Abstract
We have previously reported a group of patients with congenital onset weakness associated with a deficiency of members of the syntrophin-alpha-dystrobrevin subcomplex and have demonstrated that loss of syntrophin and dystrobrevin from the sarcolemma of skeletal muscle can also be associated with den...
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PMID: 19026398
PDF is available here.
PDF is available here.
Abstract
We used mice with targeted disruption of the gene encoding alpha-syntrophin (alpha-Syn(-/-)) that lack the perivascular AQP4 pool but retain the endothelial pool of this protein. Infarct volume at 72 h after transient focal ischemia (90 mins) in isoflurane-anesthetized mice was attenuated in both se...
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PMID: 18648381
PDF is available here.
PDF is available here.
Journal of Biological Chemistry (283)48 2008
Abstract
We show that ICA512-CCF also dimerizes with intact ICA512 on granules, thereby displacing it from beta2-syntrophin. This leads to increased granule mobility and insulin release. Based on these findings, we propose a model whereby the generation of ICA512-CCF first amplifies insulin secretion. The en...
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PMID: 18824546
PDF is available here.
PDF is available here.
Psychiatric Genetics (18)5 2008
Abstract
We found no convincing evidence for association between any of the variants studied and anxiety, depression, or neuroticism. CONCLUSION: This study sample is relatively large but our results do not support an association between BDNF Val66Met and anxiety, depression, or neuroticisim. DTNBP1 haplotyp...
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PMID: 18797396
PDF is available here.
PDF is available here.
Journal of Biological Chemistry (283)27 2008
Abstract
We identified the dystrophin proteins, syntrophin, dystrobrevin, and utrophin as essential GPCR-interacting proteins for alpha(1D)-ARs. We found that dystrophins complex with alpha(1D)-AR both in vitro and in vivo to ensure proper functional expression. More importantly, we demonstrate that knock-ou...
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PMID: 18468998
PDF is available here.
PDF is available here.
Neuroscience (154)2 2008
Abstract
We established a primary muscle cell culture system from alphaDB knockout mice and stably expressed individual alphaDB isoforms using retroviral infection. A comparison between wild-type and alphaDB knockout muscle cells showed that in the absence of alphaDB, fewer AChR clusters formed in response t...
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PMID: 18468804
PDF is available here.
PDF is available here.
Circulation (117)19 2008
Abstract
Selective vacuolar degeneration of Purkinje fibers was found in the early stages of dystrophin deficiency. Dislocation of utrophin besides upregulation of Dp71 can be involved with this pathology. The degeneration of Purkinje fibers can be associated with the distinct deep Q waves in ECG and fatal a...
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PMID: 18458171
PDF is available here.
PDF is available here.
Abstract
We define the scaffolding potential of the Schwann cell dystrophin glycoprotein complex (DGC) by establishing the presence of four syntrophin isoforms, (alpha1, beta1, beta2, and gamma2), and one dystrobrevin isoform, (alpha-dystrobrevin-1), in the abaxonal membrane. Furthermore, we demonstrate the...
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PMID: 18286648
PDF is available here.
PDF is available here.
Brain Research (1201) 2008
Abstract
We show that a subset of neurons in the hypothalamus contains alpha-DB. Comparative immunohistochemical studies with two alpha-DB antibodies of different specificity indicate that the neurons contain short alpha-DB isoform(s) alpha-DB-2 and/or alpha-DB-4. Immunoreactive multipolar or spindle-shaped...
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PMID: 18314094
PDF is available here.
PDF is available here.
Abstract
alpha-Dystrobrevin mRNA including exons 11A and 12 was increased in both skeletal and cardiac muscle of DM1 patients. The aberrantly spliced alpha-dystrobrevin isoform was localized to the sarcolemma, and showed increased binding with alpha-syntrophin. Furthermore, levels of alpha-syntrophin associa...
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PMID: 18299519
PDF is available here.
PDF is available here.
Abstract
We have investigated the biochemical properties and localization of MLC1 in cultured astrocytes and brain tissue and searched for evidence of a relationship between MLC1 and proteins of the dystrophin-glycoprotein complex (DGC). Cultured astrocytes express two MLC1 components showing different solub...
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PMID: 18165104
PDF is available here.
PDF is available here.
Neuropharmacology (54)2 2008
Abstract
We have previously shown, in mice, that treatment with the antiepileptic drug vigabatrin (GVG) on postnatal days 4-14 delays reflex development in the newborn and impairs learning and memory in the adult. Here, we report the time course in which postnatal GVG treatment induced behavioral changes in...
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PMID: 18063001
PDF is available here.
PDF is available here.
Abstract
We previously demonstrated the presence of Dp71 isoforms, utrophins, and various dystrophin-associated proteins and their participation in cytoskeleton re-organization, filopodia and lamellipodia extension, and in centralizing cytoplasmic granules during the adhesion process of human platelets. To e...
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PMID: 18180614
PDF is available here.
PDF is available here.
Journal of Cell Science (121)Pt 1 2008
Abstract
We used a transgenic approach to restore alpha-dystrobrevin to the sarcolemma in mice that lack dystrophin (mdx mice) to study two interrelated functions: (1) the ability of alpha-dystrobrevin to rescue components of the dystrophin complex in the absence of dystrophin and (2) the ability of sarcolem...
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PMID: 18057022
PDF is available here.
PDF is available here.
Biochemistry (Washington) (46)51 2007
Abstract
Binding of laminin to dystroglycan in the dystrophin glycoprotein complex causes signaling through dystroglycan-syntrophin-grb2-SOS1-Rac1-PAK1-JNK. Laminin binding also causes syntrophin tyrosine phosphorylation to initiate signaling. The kinase responsible was investigated here. PP2 and SU6656, spe...
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PMID: 18044967
PDF is available here.
PDF is available here.
Journal of Neurochemistry (103)5 2007
Abstract
We used the Large(myd) mouse, an animal model for dystroglycanopathies. We found that Kir4.1 and AQP4 are lost from astrocytic endfeet in brain whereas significant labeling for these channels is detected at similar cell domains in retina. Furthermore, while both alpha- and beta1-syntrophins are lost...
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PMID: 17803675
PDF is available here.
PDF is available here.
Neuromuscular Disorders (17)11-12 2007
Abstract
We identify further sites of syncoilin location in normal muscle: at the perinuclear space, myotendinous junction, and enrichment in the sarcolemma and sarcoplasm of oxidative muscle fibers in mice. To understand the importance of the dystrophin protein complex-syncoilin-cytoskeletal link and its im...
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PMID: 17629480
PDF is available here.
PDF is available here.
Abstract
We were therefore surprised to find that the analogous regions of the Schistosoma mansoni proteins bear multiple large insertions amounting to 100% (dystrophin) and 35% (dystrobrevin) of their expected size. We isolated orthologous sequences from Schistosoma haematobium and Schistosoma bovis, and fo...
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PMID: 17764661
PDF is available here.
PDF is available here.
Muscle & Nerve (36)4 2007
Abstract
Our findings show where synemin is expressed in brain and allow one to speculate with regard to its function in neural tissue....
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PMID: 17654553
PDF is available here.
PDF is available here.
Journal of Molecular Biology (371)5 2007
Abstract
We sought new insights and performed a two-hybrid screen of a mouse brain cDNA library using beta-dystrobrevin, the isoform expressed in non-muscle tissues, as bait. Among the positive clones characterized after the screen, one encodes the regulatory subunit RIalpha of the cAMP-dependent protein kin...
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PMID: 17610895
PDF is available here.
PDF is available here.
Abstract
We identified dystrophin associated proteins (DAPs) that associate in vivo with Dp71ab during nerve growth factor (NGF) induced differentiation of PC12 cells. DAPs expression was analyzed by RT-PCR, Western blot and indirect immunofluorescence, showing the presence of each mRNA and protein correspon...
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PMID: 17390338
PDF is available here.
PDF is available here.
Abstract
We performed a yeast two-hybrid screen and identified pancortin-2 as a novel beta-dystrobrevin-binding partner. Pancortins-1-4 are neuron-specific olfactomedin-related glycoproteins, highly expressed during brain development and widely distributed in the mature cerebral cortex of the mouse. Pancorti...
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PMID: 17265465
PDF is available here.
PDF is available here.
Abstract
We used fluorescence recovery after photobleaching, photo-unbinding, and quantitative fluorescence imaging to investigate the surface mobility and stability of AChE at the adult innervated neuromuscular junction of living mice. In wild-type synapses, we found that nonsynaptic (perisynaptic and extra...
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PMID: 17538015
PDF is available here.
PDF is available here.
Abstract
We investigated whether DGK-zeta has a role in the fusion of cultured C2C12 myoblasts. We show that DGK-zeta and syntrophins, scaffold proteins of the dystrophin glycoprotein complex that bind directly to DGK-zeta, are spatially regulated during fusion. Both proteins accumulated with the GTPase Rac1...
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PMID: 17410543
PDF is available here.
PDF is available here.
Cell Cycle (6)10 2007
Abstract
We directed our attention towards two new dipeptide boronic acid inhibitors blocking the proteasomal-dependent degradation pathway: Velcade (bortezomib or PS-341) and MLN273 (PS-273). The exciting aspect of this development is that these drugs have already progressed to preclinical and clinical tria...
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PMID: 17495527
PDF is available here.
PDF is available here.
Nippon rinsho. Japanese journal of clinical medicine (65 Suppl 4) 2007
Abstract
Abstract
Abstract
The assembly, processing and translocation of proteins occur constantly in all cells, and these processes also take place during the genesis, maintenance and repair of skeletal muscle. Skeletal muscle fibers are composed of myofibrils and are surrounded by a muscle plasma membrane, the sarcolemma. T...
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PMID: 17274800
PDF is available here.
PDF is available here.
FASEB Journal (21)2 2007
Abstract
We suggest that normal regulation of CCEs in skeletal muscle depends on the association between TRPC1 channels and alpha1-syntrophin that may anchor the store-operated channels to the dystrophin-associated protein complex (DAPC). The loss of this molecular association could participate in the calciu...
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PMID: 17202249
PDF is available here.
PDF is available here.
Neuromuscular Disorders (17)2 2007
Abstract
The alpha- and beta-dystrobrevins belong to the family of dystrophin-related and dystrophin-associated proteins. As constituents of the dystrophin-associated protein complex, alpha-dystrobrevin was believed to have a role predominantly in muscles and beta-dystrobrevin in non-muscle tissues. Recent r...
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PMID: 17251025
PDF is available here.
PDF is available here.
Gene Expression (14)1 2007
Abstract
We used a two-step homologous recombination strategy combined with in vivo Cre-mediated excision to generate mice with a large deletion of the alpha-dystrobrevin gene to disrupt all isoforms. However, these mice did not exhibit a more severe NMJ phenotype than that observed in the exon 3-deleted mic...
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PMID: 17933218
PDF is available here.
PDF is available here.
Abstract
With an anti-alpha-dystrobrevin antibody, beta-synemin co-immunoprecipitated with alpha-dystrobrevin whereas with an anti-beta-synemin antibody, alpha-dystrobrevin-1 (rather than the -2 isoform) preferentially co-immunoprecipitated with beta-synemin. Immunohistochemical experiments show that beta-sy...
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PMID: 17493272
PDF is available here.
PDF is available here.
BMC Genomics (8)1 2007
Abstract
Features of the superfamily revealed by our survey include: a) Dystrotelin, an entirely novel branch of the superfamily, present in most vertebrates examined. Dystrotelin is expressed in the central nervous system, and is a possible orthologue of Drosophila DAH. We describe the preliminary character...
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PMID: 17233888
PDF is available here.
PDF is available here.
Cell and Tissue Research (327)1 2007
Abstract
We describe previously unknown expression patterns and the localisation and molecular characteristics of alpha-DB isoforms in non-muscle mouse tissues. We demonstrate a highly specific sub-cellular distribution of alpha-DB in organs forming blood-tissue barriers. We show alpha-DB expression and loca...
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PMID: 16868787
PDF is available here.
PDF is available here.
Abstract
We present a case of INVM (left and right ventricles) in a 3-year-old girl, diagnosed by two-dimensional echocardiography. The anomaly presented as a restrictive cardiomyopathy. The girl was admitted to our hospital with heart failure, when she was 10 months old. She was treated with dopamine, digox...
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PMID: 17101628
PDF is available here.
PDF is available here.