Enhanced expression of the α7β1 integrin reduces muscular dystrophy and restores viability in dystrophic mice

Journal of Cell Biology

Volumn 152, Issue 6, 2001, Pages 1207-1218

  Burkin, Dean J ^a   Wallace, Gregory Q ^a   Nicol, Kimberly J ^a   Kaufman, David J ^b   Kaufman, Stephen J ^a  

^a UNIVERSITY OF ILLINOIS AT URBANA CHAMPAIGN   (United States)

^b NATIONAL CANCER INSTITUTE   (United States)

Author keywords

7 1 integrin; Dystrophin; Muscular dystrophy; Neuromuscular junction; Utrophin

Indexed keywords

CREATINE KINASE; DYSTROPHIN; INTEGRIN; LAMININ; UTROPHIN;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; CYTOSKELETON; DUCHENNE MUSCULAR DYSTROPHY; ELECTRON MICROSCOPY; EXTRACELLULAR MATRIX; GAIT; GENE EXPRESSION; IMMUNOFLUORESCENCE; JOINT CONTRACTURE; KYPHOSIS; LONGEVITY; MOUSE; MUSCULAR DYSTROPHY; NONHUMAN; PRIORITY JOURNAL; PROMOTER REGION; SOUTHERN BLOTTING; TRANSGENIC MOUSE; WESTERN BLOTTING;

ANIMALS; BLOTTING, WESTERN; BODY WEIGHT; CONTRACTURE; CREATINE KINASE; CREATINE KINASE, MM FORM; CYTOSKELETAL PROTEINS; DYSTROPHIN; FEMALE; HINDLIMB; HUMANS; INTEGRINS; ISOENZYMES; JOINTS; KYPHOSIS; MAGNETIC RESONANCE IMAGING; MALE; MEMBRANE PROTEINS; MICE; MICE, INBRED MDX; MICE, TRANSGENIC; MICROSCOPY, FLUORESCENCE; MUSCLE, SKELETAL; MUSCULAR DYSTROPHY, ANIMAL; MUSCULAR DYSTROPHY, DUCHENNE; NEUROMUSCULAR JUNCTION; PROMOTER REGIONS (GENETICS); RATS; RECEPTORS, CHOLINERGIC; SURVIVAL RATE; TRANSGENES; UTROPHIN;

ANIMALIA; MUS MUSCULUS; TRIXIS;

EID: 0035911960     PISSN: 00219525     EISSN: None     Source Type: Journal    
DOI: 10.1083/jcb.152.6.1207     Document Type: Article

References (59)

1. The mdx-amplification- resistant mutation system assay, a simple and rapid polymerase chain reaction-based detection of the mdx allele
2. Beta 1D integrin displaces the beta 1A isoform in striated muscles: Localization at junctional structures and signaling potential in nonmuscle cells
3. Muscle beta1D integrin reinforces the cytoskeleton-matrix link: Modulation of integrin adhesive function by alternative splicing
4. Muscle calcium and magnesium content in Duchenne muscular dystrophy
5. X chromosome-linked muscular dystrophy (mdx) in the mouse
6. The α7β1 integrin in muscle development and disease
7. A functional role for specific spliced variants of the α7β1 integrin in acetylcholine receptor clustering
8. Laminin and α7β1 integrin regulate agrin-induced clustering of acetylcholine receptors
9. Three muscular dystrophies: Loss of cytoskeleton-extracellular matrix linkage
10. A new isoform of the laminin receptor integrin alpha 7 beta 1 is developmentally regulated in skeletal muscle
11. Laminin polymerization induces a receptor-cytoskeleton network
12. Postsynaptic abnormalities at the neuromuscular junctions of utrophin-deficient mice
13. Utrophin-dystrophin deficient mice as a model for Duchenne muscular dystrophy
14. Myotubes from transgenic mdx mice expressing full-length dystrophin show normal calcium regulation
15. Fiber regeneration is not persistent in dystrophic (mdx) mouse skeletal muscle
16. Analysis of muscle creatine kinase gene regulatory elements in skeletal and cardiac muscles of transgenic mice
17. Subtle neuromuscular defects in utrophin-deficient mice
18. Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: A model for Duchenne muscular dystrophy
19. Role for alpha-dystrobrevin in the pathogenesis of dystrophin-dependent muscular dystrophies
20. Abnormal localization of laminin subunits in muscular dystrophies
21. Mutations in the integrin α7 gene cause congenital myopathy
22. Developmental regulation and functional significance of alternative splicing of NCAM and α7β1 integrin in skeletal muscle
23. Altered expression of the α7β1 integrin in human and murine muscular dystrophies
24. Integrins: Versatility, modulation, and signaling in cell adhesion
25. Transcriptional regulation of the muscle creatine kinase gene and related expression in transfected mouse myoblasts
26. Muscle creatine kinase sequence elements regulating skeletal and cardiac muscle expression in transgenic mice
27. Nonparametric estimation from incomplete observations
28. Cellular localization of α3β1 integrin isoforms in association with myofibril-logenesis during cardiac myocyte development in culture
29. Calreticulin couples calcium release and calcium influx in α7β1 integrin-mediated calcium signaling
30. Talin, vinculin and DRP (utrophin) concentrations are increased at the mdx myotendinous junctions following onset of necrosis
31. A novel extracellular domain variant of the human integrin α7 subunit generated by alternative intron splicing
32. The sarcoglycan complex in limb-girdle muscular dystrophy
33. Synaptic integrins: Selective association of the α1 and α7A, and β7B subunits with the neuromuscular junction
34. Regenerating adult chicken skeletal muscle and satellite cell cultures express embryonic patterns of myosin and tropomyosin isoforms
35. Dystrophin-glycoprotein complex: Its role in the molecular pathogenesis of muscular dystrophies
36. Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle
37. Absence of integrin alpha 7 causes a novel form of muscular dystrophy
38. Isolation of candidate cDNAs for portions of the Duchenne muscular dystrophy gene
39. Longitudinal study of spinal deformity in Duchenne muscular dystrophy
40. Design and analysis of randomized clinical trials requiring prolonged observation of each patient. II. Analysis and examples
41. Does utrophin expression in muscles of mdx mice during postnatal development functionally compensate for dystrophin deficiency
42. Skeletal muscle-specific expression of a utrophin transgene rescues utrophin-dystrophin deficient mice
43. Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure
44. Immunochemical analysis of protein isoforms in thick myofilaments of regenerating skeletal muscle
45. Activation of c-Raf-1 kinase signal transduction pathway in alpha(7) integrin-deficient mice
46. E-box sites and a proximal regulatory region of the muscle creatine kinase gene differentially regulate expression in diverse skeletal muscles and cardiac muscle of transgenic mice
47. The molecular basis of muscular dystrophy in the mdx mouse: A point mutation
48. H36-α7 is a novel integrin alpha chain that is developmentally regulated during skeletal myogenesis
49. Expression of α7 integrin cytoplasmic domains during skeletal muscle development: Alternate forms, conformational change, and homologies with serine/threonine kinases and tyrosine phosphatases
50. Amelioration of the dystrophic phenotype of mdx mice using a truncated utrophin transgene
51. Increased protein degradation results from elevated free calcium levels found in muscle from mdx mice
52. A novel beta 1 integrin isoform produced by alternative splicing: Unique expression in cardiac and skeletal muscle
53. Structure, genetic localization, and identification of the cardiac and skeletal muscle transcripts of the human integrin alpha 7 gene (ITGA)
54. Skeletal myoblasts utilize a novel al-series integrin and not α6β1 for binding to the E8 and T8 fragments of laminin
55. Localization of the α7 integrin gene (ITGA7) on human chromosome 12q13: Clustering of integrin and hox genes implies parallel evolution of these gene families
56. Novel isoform of beta 1 integrin expressed in skeletal and cardiac muscle
57. Alternative extracellular and cytoplasmic domains of the integrin α7 subunit are differentially expressed during development