Nrap

Summary

Gene Symbol: Nrap
Description: nebulin-related anchoring protein
Alias: nebulin-related-anchoring protein, N-RAP, nrap
Species: mouse
Products:     Nrap

Top Publications

  1. Luo G, Zhang J, Nguyen T, Herrera A, Paterson B, Horowits R. Complete cDNA sequence and tissue localization of N-RAP, a novel nebulin-related protein of striated muscle. Cell Motil Cytoskeleton. 1997;38:75-90 pubmed
  2. Mohiddin S, Lu S, Cardoso J, Carroll S, Jha S, Horowits R, et al. Genomic organization, alternative splicing, and expression of human and mouse N-RAP, a nebulin-related LIM protein of striated muscle. Cell Motil Cytoskeleton. 2003;55:200-12 pubmed
    ..N-RAP is highly conserved and exclusively expressed in cardiac and skeletal muscle. Genetic abnormalities remain excellent candidate causes for cardiac and skeletal myopathies. ..
  3. Lin X, Miller J, Mankodi A, Kanadia R, Yuan Y, Moxley R, et al. Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Hum Mol Genet. 2006;15:2087-97 pubmed
    ..Sequestration of MBNL1, and failure to maintain these splicing transitions, has a pivotal role in the pathogenesis of muscle disease in DM. ..
  4. Gladman J, Mandal M, Srinivasan V, Mahadevan M. Age of onset of RNA toxicity influences phenotypic severity: evidence from an inducible mouse model of myotonic dystrophy (DM1). PLoS ONE. 2013;8:e72907 pubmed publisher
    ..Furthermore, these results also represent the first murine model for early-onset DM1 and provide a tool to investigate the effects of RNA toxicity at various stages of development. ..
  5. Luo G, Leroy E, Kozak C, Polymeropoulos M, Horowits R. Mapping of the gene (NRAP) encoding N-RAP in the mouse and human genomes. Genomics. 1997;45:229-32 pubmed
    ..We mapped the NRAP gene to mouse chromosome 19 using interspecific crosses and to human chromosome 10 using radiation hybrid panels...
  6. Luo G, Herrera A, Horowits R. Molecular interactions of N-RAP, a nebulin-related protein of striated muscle myotendon junctions and intercalated disks. Biochemistry. 1999;38:6135-43 pubmed
  7. Lu S, Carroll S, Herrera A, Ozanne B, Horowits R. New N-RAP-binding partners alpha-actinin, filamin and Krp1 detected by yeast two-hybrid screening: implications for myofibril assembly. J Cell Sci. 2003;116:2169-78 pubmed
    ..The results suggest that sequential recruitment of N-RAP binding partners may serve an important role during myofibril assembly. ..
  8. Carroll S, Lu S, Herrera A, Horowits R. N-RAP scaffolds I-Z-I assembly during myofibrillogenesis in cultured chick cardiomyocytes. J Cell Sci. 2004;117:105-14 pubmed
    ..This work strongly supports the N-RAP scaffolding model of premyofibril assembly. ..
  9. Risebro C, Searles R, Melville A, Ehler E, Jina N, Shah S, et al. Prox1 maintains muscle structure and growth in the developing heart. Development. 2009;136:495-505 pubmed publisher
  10. Krebs L, Bradley C, Norton C, Xu J, Oram K, Starling C, et al. The Notch-regulated ankyrin repeat protein is required for proper anterior-posterior somite patterning in mice. Genesis. 2012;50:366-74 pubmed publisher

Detail Information

Publications15

  1. Luo G, Zhang J, Nguyen T, Herrera A, Paterson B, Horowits R. Complete cDNA sequence and tissue localization of N-RAP, a novel nebulin-related protein of striated muscle. Cell Motil Cytoskeleton. 1997;38:75-90 pubmed
  2. Mohiddin S, Lu S, Cardoso J, Carroll S, Jha S, Horowits R, et al. Genomic organization, alternative splicing, and expression of human and mouse N-RAP, a nebulin-related LIM protein of striated muscle. Cell Motil Cytoskeleton. 2003;55:200-12 pubmed
    ..N-RAP is highly conserved and exclusively expressed in cardiac and skeletal muscle. Genetic abnormalities remain excellent candidate causes for cardiac and skeletal myopathies. ..
  3. Lin X, Miller J, Mankodi A, Kanadia R, Yuan Y, Moxley R, et al. Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Hum Mol Genet. 2006;15:2087-97 pubmed
    ..Sequestration of MBNL1, and failure to maintain these splicing transitions, has a pivotal role in the pathogenesis of muscle disease in DM. ..
  4. Gladman J, Mandal M, Srinivasan V, Mahadevan M. Age of onset of RNA toxicity influences phenotypic severity: evidence from an inducible mouse model of myotonic dystrophy (DM1). PLoS ONE. 2013;8:e72907 pubmed publisher
    ..Furthermore, these results also represent the first murine model for early-onset DM1 and provide a tool to investigate the effects of RNA toxicity at various stages of development. ..
  5. Luo G, Leroy E, Kozak C, Polymeropoulos M, Horowits R. Mapping of the gene (NRAP) encoding N-RAP in the mouse and human genomes. Genomics. 1997;45:229-32 pubmed
    ..We mapped the NRAP gene to mouse chromosome 19 using interspecific crosses and to human chromosome 10 using radiation hybrid panels...
  6. Luo G, Herrera A, Horowits R. Molecular interactions of N-RAP, a nebulin-related protein of striated muscle myotendon junctions and intercalated disks. Biochemistry. 1999;38:6135-43 pubmed
  7. Lu S, Carroll S, Herrera A, Ozanne B, Horowits R. New N-RAP-binding partners alpha-actinin, filamin and Krp1 detected by yeast two-hybrid screening: implications for myofibril assembly. J Cell Sci. 2003;116:2169-78 pubmed
    ..The results suggest that sequential recruitment of N-RAP binding partners may serve an important role during myofibril assembly. ..
  8. Carroll S, Lu S, Herrera A, Horowits R. N-RAP scaffolds I-Z-I assembly during myofibrillogenesis in cultured chick cardiomyocytes. J Cell Sci. 2004;117:105-14 pubmed
    ..This work strongly supports the N-RAP scaffolding model of premyofibril assembly. ..
  9. Risebro C, Searles R, Melville A, Ehler E, Jina N, Shah S, et al. Prox1 maintains muscle structure and growth in the developing heart. Development. 2009;136:495-505 pubmed publisher
  10. Krebs L, Bradley C, Norton C, Xu J, Oram K, Starling C, et al. The Notch-regulated ankyrin repeat protein is required for proper anterior-posterior somite patterning in mice. Genesis. 2012;50:366-74 pubmed publisher
  11. Zhang J, Elzey B, Williams G, Lu S, Law D, Horowits R. Ultrastructural and biochemical localization of N-RAP at the interface between myofibrils and intercalated disks in the mouse heart. Biochemistry. 2001;40:14898-906 pubmed
    ..These results demonstrate that N-RAP remains tightly bound to myofibrils and fasciae adherentes during biochemical purification and may be a key constituent in the mechanical link between these two structures. ..
  12. Lu S, Borst D, Horowits R. N-RAP expression during mouse heart development. Dev Dyn. 2005;233:201-12 pubmed
    ..The results are consistent with N-RAP functioning as a catalytic scaffolding molecule, with low levels of the scaffold being sufficient to repetitively catalyze key steps in myofibril assembly. ..
  13. Lu S, Borst D, Horowits R. Expression and alternative splicing of N-RAP during mouse skeletal muscle development. Cell Motil Cytoskeleton. 2008;65:945-54 pubmed publisher
    ..The results establish that N-RAP-s is the predominant spliced form of N-RAP present throughout skeletal muscle development. ..
  14. Lu S, Crawford G, Dore J, Anderson S, Despres D, Horowits R. Cardiac-specific NRAP overexpression causes right ventricular dysfunction in mice. Exp Cell Res. 2011;317:1226-37 pubmed publisher
    The muscle-specific protein NRAP is concentrated at cardiac intercalated disks, plays a role in myofibril assembly, and is upregulated early in mouse models of dilated cardiomyopathy...
  15. Garg A, O Rourke J, Long C, Doering J, Ravenscroft G, Bezprozvannaya S, et al. KLHL40 deficiency destabilizes thin filament proteins and promotes nemaline myopathy. J Clin Invest. 2014;124:3529-39 pubmed publisher
    ..Loss of sarcomere thin filament proteins is a frequent cause of NM; therefore, our data that KLHL40 stabilizes NEB and LMOD3 provide a potential basis for the development of NM in KLHL40-deficient patients. ..