Gene Symbol: Dtna
Description: dystrobrevin alpha
Alias: 2210407P21Rik, DTN-A, Dtn, Gm19389, a-DB-1, adbn, dystrobrevin alpha, alpha-dystrobrevin
Species: mouse
Products:     Dtna

Top Publications

  1. Grady R, Zhou H, Cunningham J, Henry M, Campbell K, Sanes J. Maturation and maintenance of the neuromuscular synapse: genetic evidence for roles of the dystrophin--glycoprotein complex. Neuron. 2000;25:279-93 pubmed
    ..Together, these results demonstrate involvement of distinct protein complexes in the formation and maintenance of the synapse and implicate the DGC in the latter process. ..
  2. Grady R, Grange R, Lau K, Maimone M, Nichol M, Stull J, et al. Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies. Nat Cell Biol. 1999;1:215-20 pubmed
    ..These results indicate that both signalling and structural functions of the DGC are required for muscle stability, and implicate alpha-dystrobrevin in the former...
  3. Hazai D, Lien C, Hajos F, Halasy K, Gorecki D, Jancsik V. Synaptic alpha-dystrobrevin: localization of a short alpha-dystrobrevin isoform in melanin-concentrating hormone neurons of the hypothalamus. Brain Res. 2008;1201:52-9 pubmed publisher
    ..We propose that alpha-DB plays a role in a structure or regulation mechanism unique to MCH-expressing neurons. ..
  4. Grady R, Wozniak D, Ohlemiller K, Sanes J. Cerebellar synaptic defects and abnormal motor behavior in mice lacking alpha- and beta-dystrobrevin. J Neurosci. 2006;26:2841-51 pubmed
    ..We suggest that motor deficits in muscular dystrophy patients, which are their cardinal symptoms, may reflect not only peripheral derangements but also CNS defects. ..
  5. Ambrose H, Blake D, Nawrotzki R, Davies K. Genomic organization of the mouse dystrobrevin gene: comparative analysis with the dystrophin gene. Genomics. 1997;39:359-69 pubmed
    ..Interestingly, although there is only 27% amino acid identity between the homologous regions of dystrobrevin and dystrophin, the positions of 8 of the 15 exon-intron junctions are identical. ..
  6. Nawrotzki R, Loh N, Ruegg M, Davies K, Blake D. Characterisation of alpha-dystrobrevin in muscle. J Cell Sci. 1998;111 ( Pt 17):2595-605 pubmed
  7. Adams M, Kramarcy N, Krall S, Rossi S, Rotundo R, Sealock R, et al. Absence of alpha-syntrophin leads to structurally aberrant neuromuscular synapses deficient in utrophin. J Cell Biol. 2000;150:1385-98 pubmed
    ..Thus, alpha-syntrophin has an important role in synapse formation and in the organization of utrophin, acetylcholine receptor, and acetylcholinesterase at the neuromuscular synapse. ..
  8. Martinez Pena y Valenzuela I, Akaaboune M. Acetylcholinesterase mobility and stability at the neuromuscular junction of living mice. Mol Biol Cell. 2007;18:2904-11 pubmed
    ..These results demonstrate that nonsynaptic AChEs are mobile, whereas synaptic AChEs are more stable, and that alpha-dystrobrevin is important for controlling the density and stability of AChEs at neuromuscular synapses. ..
  9. Pawlikowski B, Maimone M. Formation of complex AChR aggregates in vitro requires alpha-dystrobrevin. Dev Neurobiol. 2009;69:326-38 pubmed publisher

More Information


  1. Peters M, Adams M, Froehner S. Differential association of syntrophin pairs with the dystrophin complex. J Cell Biol. 1997;138:81-93 pubmed
    ..Since individual syntrophins do not have intrinsic binding specificity for dystrophin, dystrobrevin, or utrophin, the observed preferential pairing of syntrophins must depend on extrinsic regulatory mechanisms. ..
  2. Benson M, Newey S, Martin Rendon E, Hawkes R, Blake D. Dysbindin, a novel coiled-coil-containing protein that interacts with the dystrobrevins in muscle and brain. J Biol Chem. 2001;276:24232-41 pubmed
    ..These findings have implications for the molecular pathology of Duchenne muscular dystrophy and may provide an alternative route for anchoring dystrobrevin and the DPC to the muscle membrane. ..
  3. Grady R, Akaaboune M, Cohen A, Maimone M, Lichtman J, Sanes J. Tyrosine-phosphorylated and nonphosphorylated isoforms of alpha-dystrobrevin: roles in skeletal muscle and its neuromuscular and myotendinous junctions. J Cell Biol. 2003;160:741-52 pubmed
    ..More generally, alphaDB may play multiple roles in muscle by means of differential distribution of isoforms with distinct signaling or structural properties...
  4. Patton B, Cunningham J, Thyboll J, Kortesmaa J, Westerblad H, Edstrom L, et al. Properly formed but improperly localized synaptic specializations in the absence of laminin alpha4. Nat Neurosci. 2001;4:597-604 pubmed
    ..Thus, formation and localization of synaptic specializations are regulated separately, and alpha4beta2gamma1 (called laminin-9) is critical in the latter process. ..
  5. Lien C, Vlachouli C, Blake D, Simons J, Gorecki D. Differential spatio-temporal expression of alpha-dystrobrevin-1 during mouse development. Gene Expr Patterns. 2004;4:583-93 pubmed
    ..In contrast, this specific expression at the induction phase decreased/disappeared at later stages of development. ..
  6. Madhavan R, Jarrett H. Interactions between dystrophin glycoprotein complex proteins. Biochemistry. 1995;34:12204-9 pubmed
    ..The interactions identified here were used to refine the existing model of DGC organization to make it consistent with the current data. ..
  7. Blake D, Hawkes R, Benson M, Beesley P. Different dystrophin-like complexes are expressed in neurons and glia. J Cell Biol. 1999;147:645-58 pubmed
    ..Thus, despite the similarity in primary sequence, alpha- and beta-dystrobrevin are differentially distributed in the brain where they form separate DPC-like complexes. ..
  8. Enigk R, Maimone M. Differential expression and developmental regulation of a novel alpha-dystrobrevin isoform in muscle. Gene. 1999;238:479-88 pubmed
  9. Blake D, Nawrotzki R, Peters M, Froehner S, Davies K. Isoform diversity of dystrobrevin, the murine 87-kDa postsynaptic protein. J Biol Chem. 1996;271:7802-10 pubmed
    ..The occurrence of many dystrobrevin isoforms is significant because alternative splicing and phosphorylation often have profound effects upon the biological activity of synaptic proteins. ..
  10. Roberts R, Bobrow M. Dystrophins in vertebrates and invertebrates. Hum Mol Genet. 1998;7:589-95 pubmed
  11. Oh H, Abraham L, van Hengel J, Stove C, Proszynski T, Gevaert K, et al. Interaction of ?-catulin with dystrobrevin contributes to integrity of dystrophin complex in muscle. J Biol Chem. 2012;287:21717-28 pubmed publisher
    ..elegans and mammalian muscles and strongly suggests that this interaction contributes to the integrity of the dystrophin complex. ..
  12. Benson M, Tinsley C, Blake D. Myospryn is a novel binding partner for dysbindin in muscle. J Biol Chem. 2004;279:10450-8 pubmed
    ..These data demonstrate for the first time that there are tissue-specific ligands for dysbindin that may play important roles in the different disease states involving this protein. ..
  13. Lyssand J, DeFino M, Tang X, Hertz A, Feller D, Wacker J, et al. Blood pressure is regulated by an alpha1D-adrenergic receptor/dystrophin signalosome. J Biol Chem. 2008;283:18792-800 pubmed publisher
    ..Our findings demonstrate that syntrophin and utrophin associate with alpha(1D)-ARs to create a functional signalosome, which is essential for alpha(1D)-AR regulation of vascular tone and blood pressure. ..
  14. Zhang K, Xiang M, Zhou L, Liu J, Curry N, Heine Suñer D, et al. Gene network and familial analyses uncover a gene network involving Tbx5/Osr1/Pcsk6 interaction in the second heart field for atrial septation. Hum Mol Genet. 2016;25:1140-51 pubmed publisher
    ..In summary, our study implicated a gene network involving Tbx5, Osr1 and Pcsk6 interaction in SHF for atrial septation, providing a molecular framework for understanding the role of Tbx5 in CHD ontogeny. ..
  15. Mercado M, Amenta A, Hagiwara H, Rafii M, Lechner B, Owens R, et al. Biglycan regulates the expression and sarcolemmal localization of dystrobrevin, syntrophin, and nNOS. FASEB J. 2006;20:1724-6 pubmed
  16. Bragg A, Amiry Moghaddam M, Ottersen O, Adams M, Froehner S. Assembly of a perivascular astrocyte protein scaffold at the mammalian blood-brain barrier is dependent on alpha-syntrophin. Glia. 2006;53:879-90 pubmed
  17. Gingras J, Gawor M, Bernadzki K, Grady R, Hallock P, Glass D, et al. Α-Dystrobrevin-1 recruits Grb2 and α-catulin to organize neurotransmitter receptors at the neuromuscular junction. J Cell Sci. 2016;129:898-911 pubmed publisher
  18. Albrecht D, Froehner S. DAMAGE, a novel alpha-dystrobrevin-associated MAGE protein in dystrophin complexes. J Biol Chem. 2004;279:7014-23 pubmed
    ..These results expand the role of dystrobrevin and the dystrophin complex in membrane signaling and disease. ..
  19. Lien C, Mohanta S, Frontczak Baniewicz M, Swinny J, Zablocka B, Gorecki D. Absence of glial ?-dystrobrevin causes abnormalities of the blood-brain barrier and progressive brain edema. J Biol Chem. 2012;287:41374-85 pubmed publisher
    ..Thus, ?-DB emerges as a central organizer of dystrophin-associated protein in glial endfeet and a rare example of a glial protein with a role in maintaining BBB function. Its abnormalities might therefore lead to BBB dysfunction...
  20. Huang W, Garcia Zepeda E, Sauty A, Oettgen H, Rothenberg M, Luster A. Molecular and biological characterization of the murine leukotriene B4 receptor expressed on eosinophils. J Exp Med. 1998;188:1063-74 pubmed
    ..Our findings indicate that this murine receptor is an LTB4 receptor that is highly expressed on activated leukocytes, including eosinophils, and may play an important role in mediating eosinophil recruitment into inflammatory foci. ..
  21. Schmidt N, Akaaboune M, Gajendran N, Martinez Pena y Valenzuela I, Wakefield S, Thurnheer R, et al. Neuregulin/ErbB regulate neuromuscular junction development by phosphorylation of ?-dystrobrevin. J Cell Biol. 2011;195:1171-84 pubmed publisher
    ..Our data indicate that NRG/ErbB signaling maintains high efficacy of synaptic transmission by stabilizing the postsynaptic apparatus via phosphorylation of ?-dystrobrevin1. ..
  22. Ceccarini M, Torreri P, Lombardi D, Macchia G, Macioce P, Petrucci T. Molecular basis of dystrobrevin interaction with kinesin heavy chain: structural determinants of their binding. J Mol Biol. 2005;354:872-82 pubmed
    ..Phosphorylation events could have a regulatory role in this context. ..
  23. Albrecht D, Sherman D, Brophy P, Froehner S. The ABCA1 cholesterol transporter associates with one of two distinct dystrophin-based scaffolds in Schwann cells. Glia. 2008;56:611-8 pubmed publisher
    ..Specifically, the cholesterol transporter ABCA1, associates with the Dp116/syntrophin complex in Cajal bands and is excluded from the DRP2/periaxin rich plaques...
  24. Pawlikowski B, Maimone M. alpha-Dystrobrevin isoforms differ in their colocalization with and stabilization of agrin-induced acetylcholine receptor clusters. Neuroscience. 2008;154:582-94 pubmed publisher
  25. Martinez Pena y Valenzuela I, Mouslim C, Pires Oliveira M, Adams M, Froehner S, Akaaboune M. Nicotinic acetylcholine receptor stability at the NMJ deficient in ?-syntrophin in vivo. J Neurosci. 2011;31:15586-96 pubmed publisher
    ..These results demonstrate that ?-syn is required for the maturation and stability of the postsynaptic apparatus and suggest that ?-syn may act via ?-dbn1. ..
  26. Wang D, Kelly B, Albrecht D, Adams M, Froehner S, Feng G. Complete deletion of all alpha-dystrobrevin isoforms does not reveal new neuromuscular junction phenotype. Gene Expr. 2007;14:47-57 pubmed
    ..This work also demonstrates a successful strategy to achieve deletion of a large genomic sequence, which can be a valuable tool for functional studies of genes encoding multiple isoforms that span a large genomic region. ..
  27. Kobayashi Y, Rader E, Crawford R, Iyengar N, Thedens D, Faulkner J, et al. Sarcolemma-localized nNOS is required to maintain activity after mild exercise. Nature. 2008;456:511-5 pubmed publisher
    ..Our results suggest that patients with an exaggerated fatigue response to mild exercise would show clinical improvement in response to treatment strategies aimed at improving exercise-induced signalling...
  28. Lyssand J, Whiting J, Lee K, Kastl R, Wacker J, Bruchas M, et al. Alpha-dystrobrevin-1 recruits alpha-catulin to the alpha1D-adrenergic receptor/dystrophin-associated protein complex signalosome. Proc Natl Acad Sci U S A. 2010;107:21854-9 pubmed publisher
    ..Taken together, our study implicates ?-catulin as a unique regulator of GPCR signaling and represents a unique expansion of the intricate and continually evolving array of GPCR signaling networks. ..
  29. Bunnell T, Jaeger M, Fitzsimons D, Prins K, Ervasti J. Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle. PLoS ONE. 2008;3:e2604 pubmed publisher
    ..Mice deficient for alpha-dystrobrevin (adbn(-/-)) exhibit extensive myofiber degeneration and neuromuscular junction abnormalities...
  30. Bragg A, Das S, Froehner S. Dystrophin-associated protein scaffolding in brain requires alpha-dystrobrevin. Neuroreport. 2010;21:695-9 pubmed publisher
    ..This is the first demonstration in which assembly of an astroglial protein scaffold containing syntrophin and dystrophin in perivascular astrocytes is dependent on the presence of alpha-dystrobrevin. ..
  31. Holzfeind P, Ambrose H, Newey S, Nawrotzki R, Blake D, Davies K. Tissue-selective expression of alpha-dystrobrevin is determined by multiple promoters. J Biol Chem. 1999;274:6250-8 pubmed
  32. Bohm S, Constantinou P, Tan S, Jin H, Roberts R. Profound human/mouse differences in alpha-dystrobrevin isoforms: a novel syntrophin-binding site and promoter missing in mouse and rat. BMC Biol. 2009;7:85 pubmed publisher
  33. Aguilar A, Wagstaff K, Suárez Sánchez R, Zinker S, Jans D, Cisneros B. Nuclear localization of the dystrophin-associated protein α-dystrobrevin through importin α2/β1 is critical for interaction with the nuclear lamina/maintenance of nuclear integrity. FASEB J. 2015;29:1842-58 pubmed publisher
    ..Because α-DB interacts specifically with lamin B1 in vivo and in vitro, nuclear α-DB would appear to play a key role in nuclear shape maintenance through association with the nuclear lamina. ..
  34. Adams M, Tesch Y, Percival J, Albrecht D, Conhaim J, Anderson K, et al. Differential targeting of nNOS and AQP4 to dystrophin-deficient sarcolemma by membrane-directed alpha-dystrobrevin. J Cell Sci. 2008;121:48-54 pubmed
    ..Thus, although nNOS and AQP4 both require interaction with the PDZ domain of alpha-syntrophin for sarcolemmal association, their localization is regulated differentially...
  35. Hanft L, Bogan D, Mayer U, Kaufman S, Kornegay J, Ervasti J. Cytoplasmic gamma-actin expression in diverse animal models of muscular dystrophy. Neuromuscul Disord. 2007;17:569-74 pubmed
    ..These data suggest that elevated gamma(cyto)-actin is part of a compensatory cytoskeletal remodeling program that may partially stabilize dystrophic muscle in some cases where the dystrophin-glycoprotein complex is compromised...
  36. Strakova J, Dean J, Sharpe K, Meyers T, Odom G, Townsend D. Dystrobrevin increases dystrophin's binding to the dystrophin-glycoprotein complex and provides protection during cardiac stress. J Mol Cell Cardiol. 2014;76:106-15 pubmed publisher
    ..Cardiac stress testing demonstrated significant heart disease in α-DB null (adbn(-/-)) mice, which displayed mortality and lesion sizes that were equivalent to those seen in dystrophin-deficient ..