| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| The absence of dystrophin leads to a dramatic reduction of the dystrophin associated proteins ( 156DAG , 59DAP , 50DAG , 43DAG and 35DAG ) in the sarcolemma of patients with Duchenne muscular dystrophy and mdx mice . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| The expression of the 43 kDa dystrophin associated glycoprotein ( 43DAG ) has been studied using immunohistochemical labelling with a monoclonal antibody , MANDAG 1 , and compared with immunolabelling for dystrophin and the dystrophin related protein , utrophin , in normal muscle and in muscle from 50 patients with neuromuscular disease . 43DAG and dystrophin were expressed in vascular smooth muscle and at the sarcolemma of normal muscle fibres , with increased labelling at neuromuscular and myotendinous junctions . 43DAG expression was reduced in Duchenne and Becker dystrophies with patchy labelling , more intense around presumptive satellite cells . ^^^ In Becker dystrophy , 43DAG expression was more extensive around individual fibres , showed more interfibre variation and was more closely related to the intensity of immunolabelling for both dystrophin and utrophin than in Duchenne dystrophy . ^^^ The results suggest that in the absence of dystrophin , 43DAG is synthesized but is not stabilized in the sarcolemma . ^^^ Stability is greater in Becker dystrophy but a normal dystrophin molecule appears to be required for the complete and stable membrane integration of 43DAG . ^^^ Utrophin may confer some additional stability to the membrane integration of 43DAG but this is incomplete where dystrophin is absent or abnormal . . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunofluorescence and immunogold labelling were used to localise the 43 kDa dystrophin associated glycoprotein ( 43DAG ) of the dystrophin glycoprotein complex in control and Duchenne muscular dystrophy ( DMD ) biopsies . ^^^ In DMD , where there is a 80 90 % reduction in the glycoprotein complex , a faint or locally patchy distribution of 43DAG was seen by immunofluorescence . ^^^ Measurement of nearest neighbour distances after immunogold labelling showed that in DMD the 43DAG was more dispersed , which is further evidence that dystrophin is normally involved in anchoring the DAGs in the plasma membrane . ^^^ Moreover , the distance separating adjacent 43DAG labelling sites ( 120 nm mode ) closely matched that separating dystrophin C terminal sites . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin associated glycoprotein complex is classified into two subcomplexes : the dystroglycan complex ( 156DAG and 43DAG ) and the sarcoglycan complex ( 50DAG , A3b , and 35DAG ) . ^^^ We examined muscles from five SCARMD patients and found that dystrophin and 43DAG were present in almost normal levels while 35DAG and the newly identified protein A3b in addition to 50DAG were absent or greatly reduced . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| The region of highest 43 K DAG ( 43DAG ) labelling projected beyond the AChRs by approximately 0 . 3 microns , as does that for dystrophin . ^^^ These results suggest that at the NMJ , the region of high 43DAG concentration encompasses the area of highest intensity labelling for both DRP and dystrophin . . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Dystrophin associated proteins are comprised of an extracellular glycoprotein of 156 kDa ( 156DAG ) , transmembrane glycoproteins of 50 kDa ( 50DAG ) , 43 kDa ( 43DAG ) and 35 kDa ( 35DAG ) , and a cytoskeletal protein of 59 kDa ( 59DAP ) . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| The frequency of patients with 50 kd dystrophin associated glycoprotein ( 50DAG or adhalin ) deficiency in a muscular dystrophy patient population in Japan : immunocytochemical analysis of 50DAG , 43DAG , dystrophin , and utrophin . ^^^ To elucidate the frequency of patients having the 50DAG deficiency in a muscular dystrophy population in Japan , we immunocytochemically examined 50DAG , 43DAG , dystrophin , and utrophin . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| We now present biochemical evidence for an association between utrophin ( dystrophin related protein , DRP ) and a major DAGc component , beta dystroglycan ( 43DAG ) in cultured cell lines which contain little if any dystrophin . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| A dystrophin associated glycoprotein , A3a ( one of 43DAG doublets ) , is retained in Duchenne muscular dystrophy muscle . ^^^ We determined several internal amino acid sequences of dystrophin associated glycoprotein , A3a ( one of the 43DAG doublets ) , of rabbit skeletal muscle . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| We found that , although the amount of GPC was reduced in DMD muscles where utrophin but not dystrophin was distinctly present , 43DAG ( A3a ) was fairly heavily and 50DAG ( A 2 ) was lightly but distinctly stained on the cell surfaces . ^^^ Therefore , it is likely that 43DAG ( A3a ) is essential for the fixation of utrophin to cell membranes , as in the case of dystrophin . 50DAG ( A 2 ) may play other important roles in the pathogenesis of DMD . . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Distribution of dystrophin isoforms and dystrophin associated proteins 43DAG ( A3a ) and 50DAG ( A 2 ) in various monkey tissues . ^^^ To determine the distributions of two known dystrophin isoforms derived from the 3 ' part of the dystrophin gene and of the dystrophin associated proteins [ 50DAG ( A 2 ) and 43DAG ( A3a ) ] by immunoblot analysis , we examined various monkey tissues [ skeletal ( quadriceps ) , cardiac ( left ventricle ) , and smooth ( aorta and uterus ) muscles , lung , liver , central nervous system ( cerebrum , cerebellum , and spinal cord ) , and peripheral nerve ( sciatic nerve ) ] . ^^^ Dystrophin associated protein , 43DAG , was detected in all the tissues examined , but 50DAG was detected only in skeletal and cardiac muscles . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Antibodies against dystrophin , utrophin and DAGs including 50DAG ( A 2 ) , 43DAG ( A3a ) and 35DAG ( A 4 ) were employed for the examination . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Adhalin ( 50 kDa dystrophin associated glycoprotein ) and beta dystroglycan ( 43 kDa dystrophin associated glycoprotein ) are the transmembrane components of the normal muscle plasma membrane , and beta dystroglycan has been demonstrated to bind dystrophin at the inside surface of normal muscle plasma membrane . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| The dystrophin associated proteins are classified into three groups : ( 1 ) alpha and beta dystroglycan , ( 2 ) adhalin , 35DAG and A3b , and ( 3 ) members of the syntrophin family . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Loss of the sarcoglycan complex and sarcospan leads to muscular dystrophy in beta sarcoglycan deficient mice . beta Sarcoglycan , one of the subunits of the sarcoglycan complex , is a transmembranous glycoprotein which associates with dystrophin and is the molecule responsible for beta sarcoglycanopathy , a Duchenne like autosomal recessive muscular dystrophy . ^^^ Our data suggest that loss of the sarcoglycan complex and sarcospan alone is sufficient to cause muscular dystrophy , that beta sarcoglycan is an important protein for formation of the sarcoglycan complex associated with sarcospan and that the role of the sarcoglycan complex and sarcospan may be to strengthen the dystrophin axis connecting the basement membrane with the cytoskeleton . . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| In dystrophin deficient cardiac muscle , utrophin and beta sarcoglycan were observed in intercalated disks and at the sarcolemma of each cardiocyte . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Distribution of dystrophin and dystrophin associated protein 43DAG ( beta dystroglycan ) in the central nervous system of normal controls and patients with Duchenne muscular dystrophy . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| To evaluate a potential regulatory role of the nerve , the distribution and expression of dystrophin , of beta dystroglycan ( 43DAG ) and adhalin ( 50DAG ) , two of the dystrophin associated proteins and utrophin ( dystrophin related protein or DRP ) were studied in rat muscles after 2 weeks of denervation . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunoelectron microscopic localization of C terminus of 43 kDa dystrophin associated glycoprotein in normal human skeletal myofibers . ^^^ Ultrastructural localization of the C terminus of a 43 kDa dystrophin associated glycoprotein ( 43 DAG ) and its relation to dystrophin were investigated in normal human skeletal myofibers by using single and double immunogold labelling methods . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| The binding of dystrophin to each protein was reconstituted by overlaying bacterially expressed dystrophin fusion proteins onto the blot membranes to which dystrophin associated proteins were transferred after separation by SDS / PAGE with the following results . ( a ) Among the components of the glycoprotein complex which links dystrophin to the sarcolemma , a 43 kDa dystrophin associated glycoprotein binds directly to dystrophin . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| We report a novel beta sarcoglycan gene mutation identified in a 21 year old Portuguese male with a progressive myopathy of intermediate severity , who had been misdiagnosed as Becker Muscular Dystrophy ( BMD ) based on clinical observations and muscle immunocytochemical anaylsis with dystrophin antibodies only . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Immunohistochemical analysis of the dystrophin associated proteins revealed a reduction of beta sarcoglycan and delta sarcoglycan in the sarcolemma of cardiac muscle but not skeletal muscle tissue . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| Secondary dystrophin reduction was seen in 44 % patients and correlated with beta sarcoglycan deficiency but not with functional disability . . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| RESULTS : We validate the temporal Hotelling T 2 test on muscular gene expression data from four mouse strains which were profiled at different ages : dystrophin , beta sarcoglycan and gamma sarcoglycan deficient mice , and wild type mice . ^^^ |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q16585 and P11532 |
Pubmed |
SVM Score :0.0 |
| NA |
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