{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T01:53:03Z","timestamp":1791165183914,"version":"4.1.0"},"reference-count":70,"publisher":"Rockefeller University Press","issue":"6","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2000,12,11]]},"abstract":"<jats:p>We used expression profiling to define the pathophysiological cascades involved in the progression of two muscular dystrophies with known primary biochemical defects, dystrophin deficiency (Duchenne muscular dystrophy) and \u03b1-sarcoglycan deficiency (a dystrophin-associated protein). We employed a novel protocol for expression profiling in human tissues using mixed samples of multiple patients and iterative comparisons of duplicate datasets. We found evidence for both incomplete differentiation of patient muscle, and for dedifferentiation of myofibers to alternative lineages with advancing age. One developmentally regulated gene characterized in detail, \u03b1-cardiac actin, showed abnormal persistent expression after birth in 60% of Duchenne dystrophy myofibers. The majority of myofibers (\u223c80%) remained strongly positive for this protein throughout the course of the disease. Other developmentally regulated genes that showed widespread overexpression in these muscular dystrophies included embryonic myosin heavy chain, versican, acetylcholine receptor \u03b1-1, secreted protein, acidic and rich in cysteine\/osteonectin, and thrombospondin 4. We hypothesize that the abnormal Ca2+ influx in dystrophin- and \u03b1-sarcoglycan\u2013deficient myofibers leads to altered developmental programming of developing and regenerating myofibers. The finding of upregulation of HLA-DR and factor XIIIa led to the novel identification of activated dendritic cell infiltration in dystrophic muscle; these cells mediate immune responses and likely induce microenvironmental changes in muscle. We also document a general metabolic crisis in dystrophic muscle, with large scale downregulation of nuclear-encoded mitochondrial gene expression. Finally, our expression profiling results show that primary genetic defects can be identified by a reduction in the corresponding RNA.<\/jats:p>","DOI":"10.1083\/jcb.151.6.1321","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T16:46:23Z","timestamp":1027701983000},"page":"1321-1336","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":419,"title":["Expression Profiling in the Muscular Dystrophies"],"prefix":"10.1083","volume":"151","author":[{"given":"Yi-Wen","family":"Chen","sequence":"first","affiliation":[{"name":"aResearch Center for Genetic Medicine, Children's National Medical Center, George Washington University, Washington, DC 20010"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Po","family":"Zhao","sequence":"additional","affiliation":[{"name":"aResearch Center for Genetic Medicine, Children's National Medical Center, George Washington University, Washington, DC 20010"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rehannah","family":"Borup","sequence":"additional","affiliation":[{"name":"aResearch Center for Genetic Medicine, Children's National Medical Center, George Washington University, Washington, DC 20010"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric P.","family":"Hoffman","sequence":"additional","affiliation":[{"name":"aResearch Center for Genetic Medicine, Children's National Medical Center, George Washington University, Washington, DC 20010"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2000,12,11]]},"reference":[{"key":"2023072902454776000_Albertietal1996","doi-asserted-by":"crossref","first-page":"4726","DOI":"10.1128\/iai.64.11.4726-4732.1996","article-title":"Analysis of complement C3 deposition and degradation on Klebsiella pneumoniae","volume":"64","author":"Alberti","year":"1996","journal-title":"Infect. 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Altered rate of phosphate transport","volume":"109","author":"Barbiroli","year":"1992","journal-title":"J. Neurol. Sci."},{"key":"2023072902454776000_Bonnemannetal1995","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1038\/ng1195-266","article-title":"Beta-sarcoglycan (A3b) mutations cause autosomal recessive muscular dystrophy with loss of the sarcoglycan complex","volume":"11","author":"Bonnemann","year":"1995","journal-title":"Nat. Genet."},{"key":"2023072902454776000_Brownetal1999","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1242\/jcs.112.2.209","article-title":"Dystrophic phenotype induced in vitro by antibody blockade of muscle alpha-dystroglycan-laminin interaction","volume":"112","author":"Brown","year":"1999","journal-title":"J. Cell Sci."},{"key":"2023072902454776000_Bulfieldetal1984","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1073\/pnas.81.4.1189","article-title":"X chromosome-linked muscular dystrophy (mdx) in the mouse","volume":"81","author":"Bulfield","year":"1984","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072902454776000_BurkinandKaufman1999","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1007\/s004410051279","article-title":"The alpha7beta1 integrin in muscle development and disease","volume":"296","author":"Burkin","year":"1999","journal-title":"Cell Tissue Res"},{"key":"2023072902454776000_Carpenteretal1989","first-page":"909","article-title":"Feline muscular dystrophy with dystrophin deficiency","volume":"135","author":"Carpenter","year":"1989","journal-title":"Am. J. Pathol."},{"key":"2023072902454776000_Carrinoetal1999","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1093\/ps\/78.5.769","article-title":"Dynamic expression of proteoglycans during chicken skeletal muscle development and maturation","volume":"78","author":"Carrino","year":"1999","journal-title":"Poult. Sci."},{"key":"2023072902454776000_Chaoetal1996","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1084\/jem.184.2.609","article-title":"Selective loss of sarcolemmal nitric oxide synthase in Becker muscular dystrophy","volume":"184","author":"Chao","year":"1996","journal-title":"J. Exp. Med."},{"key":"2023072902454776000_Chouetal1999","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1212\/WNL.52.5.1015","article-title":"Calpain III mutation analysis of a heterogeneous limb-girdle muscular dystrophy population","volume":"52","author":"Chou","year":"1999","journal-title":"Neurology"},{"key":"2023072902454776000_Connollyetal1998","doi-asserted-by":"crossref","first-page":"1549","DOI":"10.1002\/(SICI)1097-4598(199811)21:11<1549::AID-MUS30>3.0.CO;2-T","article-title":"Primary alpha-sarcoglycan deficiency responsive to immunosuppression over three years","volume":"21","author":"Connolly","year":"1998","journal-title":"Muscle Nerve"},{"key":"2023072902454776000_Cooperetal1988","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1038\/334154a0","article-title":"The homologue of the Duchenne locus is defective in X-linked muscular dystrophy of dogs","volume":"334","author":"Cooper","year":"1988","journal-title":"Nature"},{"key":"2023072902454776000_Cullenetal1996","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1111\/j.1365-2990.1996.tb00843.x","article-title":"Ultrastructural localization of adhalin, alpha-dystroglycan and merosin in normal and dystrophic muscle","volume":"22","author":"Cullen","year":"1996","journal-title":"Neuropathol. 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Cell"},{"key":"2023072902454776000_ErvastiandCampbell1991","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1016\/0092-8674(91)90035-W","article-title":"Membrane organization of the dystrophin\u2013glycoprotein complex","volume":"66","author":"Ervasti","year":"1991","journal-title":"Cell"},{"key":"2023072902454776000_Evankoetal1999","doi-asserted-by":"crossref","first-page":"1004","DOI":"10.1161\/01.ATV.19.4.1004","article-title":"Formation of hyaluronan- and versican-rich pericellular matrix is required for proliferation and migration of vascular smooth muscle cells","volume":"19","author":"Evanko","year":"1999","journal-title":"Arterioscler. Thromb. Vasc. 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Sci."},{"key":"2023072902454776000_Golubetal1999","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1126\/science.286.5439.531","article-title":"Molecular classification of cancerclass discovery and class prediction by gene expression monitoring","volume":"286","author":"Golub","year":"1999","journal-title":"Science"},{"key":"2023072902454776000_GorospeandHoffman1992","first-page":"794","article-title":"Duchenne muscular dystrophy","volume":"4","author":"Gorospe","year":"1992","journal-title":"Curr. Opin. Rheumatol."},{"key":"2023072902454776000_Gorospeetal1994a","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/0022-510X(94)90050-7","article-title":"A role for mast cells in the progression of Duchenne muscular dystrophy? Correlations in dystrophin-deficient humans, dogs, and mice","volume":"122","author":"Gorospe","year":"1994","journal-title":"J. Neurol. Sci."},{"key":"2023072902454776000_Gorospeetal1994b","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/0960-8966(94)90068-X","article-title":"Dystrophin-deficient myofibers are vulnerable to mast cell granule-induced necrosis","volume":"4","author":"Gorospe","year":"1994","journal-title":"Neuromuscul. Disord."},{"key":"2023072902454776000_Gorospeetal1996","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/0022-510X(95)00255-Z","article-title":"Recruitment of mast cells to muscle after mild damage","volume":"135","author":"Gorospe","year":"1996","journal-title":"J. Neurol. 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Disord."},{"key":"2023072902454776000_Griggsetal1993","doi-asserted-by":"crossref","first-page":"520","DOI":"10.1212\/WNL.43.3_Part_1.520","article-title":"Duchenne dystrophyrandomized, controlled trial of prednisone (18 months) and azathioprine (12 months)","volume":"43","author":"Griggs","year":"1993","journal-title":"Neurology"},{"key":"2023072902454776000_Hamedetal2001","article-title":"Complete sequence screening of the dystrophin cDNA in Duchenne dystrophythe most common mutation of the most mutable gene is a novel recurring duplication of exon 2","volume":"In press","author":"Hamed","year":"2001","journal-title":"Hum. Mutat"},{"key":"2023072902454776000_Hacketal1998","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1083\/jcb.142.5.1279","article-title":"\u03b3-Sarcoglycan deficiency leads to muscle membrane defects and apoptosis independent of dystrophin","volume":"142","author":"Hack","year":"1998","journal-title":"J. Cell Biol."},{"key":"2023072902454776000_Hasegawaetal1999","doi-asserted-by":"crossref","first-page":"12626","DOI":"10.1074\/jbc.274.18.12626","article-title":"Stress-activated protein kinase-3 interacts with the PDZ domain of alpha1-syntrophin. A mechanism for specific substrate recognition","volume":"274","author":"Hasegawa","year":"1999","journal-title":"J. Biol. Chem."},{"key":"2023072902454776000_Hayashietal1998","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1038\/ng0598-94","article-title":"Mutations in the integrin alpha7 gene cause congenital myopathy","volume":"19","author":"Hayashi","year":"1998","journal-title":"Nat. Genet."},{"key":"2023072902454776000_HoffmanandSchwartz1991","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/0098-2997(91)90001-3","article-title":"Dystrophin and disease","volume":"12","author":"Hoffman","year":"1991","journal-title":"Mol. Aspects Med"},{"key":"2023072902454776000_Hoffmanetal1987a","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1126\/science.3659917","article-title":"Conservation of the Duchenne muscular dystrophy gene in mice and humans","volume":"238","author":"Hoffman","year":"1987","journal-title":"Science"},{"key":"2023072902454776000_Hoffmanetal1987b","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/0092-8674(87)90579-4","article-title":"Dystrophinthe protein product of the Duchenne muscular dystrophy locus","volume":"51","author":"Hoffman","year":"1987","journal-title":"Cell"},{"key":"2023072902454776000_Ibraghimov-Beskrovnayaetal1992","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1038\/355696a0","article-title":"Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrix","volume":"355","author":"Ibraghimov-Beskrovnaya","year":"1992","journal-title":"Nature"},{"key":"2023072902454776000_JelinskyandSamson1999","doi-asserted-by":"crossref","first-page":"1486","DOI":"10.1073\/pnas.96.4.1486","article-title":"Global response of Saccharomyces cerevisiae to an alkylating agent","volume":"96","author":"Jelinsky","year":"1999","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072902454776000_Joossetal1998","doi-asserted-by":"crossref","first-page":"4212","DOI":"10.1128\/JVI.72.5.4212-4223.1998","article-title":"Transduction of dendritic cells by DNA viral vectors directs the immune response to transgene products in muscle fibers","volume":"72","author":"Jooss","year":"1998","journal-title":"J. Virol."},{"key":"2023072902454776000_Kaariainenetal2000","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/S0960-8966(99)00077-2","article-title":"Integrin and dystrophin associated adhesion protein complexes during regeneration of shearing-type muscle injury","volume":"10","author":"Kaariainen","year":"2000","journal-title":"Neuromuscul. Disord."},{"key":"2023072902454776000_Koenigetal1988","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/0092-8674(88)90383-2","article-title":"The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein","volume":"53","author":"Koenig","year":"1988","journal-title":"Cell"},{"key":"2023072902454776000_Kornegayetal1988","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1002\/mus.880111008","article-title":"Muscular dystrophy in a litter of golden retriever dogs","volume":"11","author":"Kornegay","year":"1988","journal-title":"Muscle Nerve"},{"key":"2023072902454776000_Kuznetsovetal1998","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1023\/A:1006868130002","article-title":"Impaired mitochondrial oxidative phosphorylation in skeletal muscle of the dystrophin-deficient mdx mouse","volume":"183","author":"Kuznetsov","year":"1998","journal-title":"Mol. Cell. Biochem"},{"key":"2023072902454776000_LaneandSage1994","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1096\/fasebj.8.2.8119487","article-title":"The biology of SPARC, a protein that modulates cell\u2013matrix interactions","volume":"8","author":"Lane","year":"1994","journal-title":"FASEB J."},{"key":"2023072902454776000_Lawleretal1995","doi-asserted-by":"crossref","first-page":"2809","DOI":"10.1074\/jbc.270.6.2809","article-title":"Characterization of human thrombospondin-4","volume":"270","author":"Lawler","year":"1995","journal-title":"J. Biol. Chem."},{"key":"2023072902454776000_Lockhartetal1996","doi-asserted-by":"crossref","first-page":"1675","DOI":"10.1038\/nbt1296-1675","article-title":"Expression monitoring by hybridization to high-density oligonucleotide arrays","volume":"14","author":"Lockhart","year":"1996","journal-title":"Nat. Biotechnol."},{"key":"2023072902454776000_Matasicetal1999","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1002\/jlb.66.6.909","article-title":"Dexamethasone inhibits dendritic cell maturation by redirecting differentiation of a subset of cells","volume":"66","author":"Matasic","year":"1999","journal-title":"J. Leukoc. Biol."},{"key":"2023072902454776000_McNallyetal1996","doi-asserted-by":"crossref","first-page":"1841","DOI":"10.1093\/hmg\/5.11.1841","article-title":"Mutations that disrupt the carboxyl-terminus of gamma-sarcoglycan cause muscular dystrophy","volume":"5","author":"McNally","year":"1996","journal-title":"Hum. Mol. Genet."},{"key":"2023072902454776000_Mizunoetal1995","first-page":"530","article-title":"Sarcoglycan complex is selectively lost in dystrophic hamster muscle","volume":"146","author":"Mizuno","year":"1995","journal-title":"Am. J. Pathol."},{"key":"2023072902454776000_Nayaetal2000","doi-asserted-by":"crossref","first-page":"4545","DOI":"10.1074\/jbc.275.7.4545","article-title":"Stimulation of slow skeletal muscle fiber gene expression by calcineurin in vivo","volume":"275","author":"Naya","year":"2000","journal-title":"J. Biol. Chem."},{"key":"2023072902454776000_Newtonetal1999","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1007\/s003359901149","article-title":"The thrombospondin-4 gene","volume":"10","author":"Newton","year":"1999","journal-title":"Mamm. Genome"},{"key":"2023072902454776000_Nicholsonetal1989","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/0022-510X(89)90224-4","article-title":"Dystrophin in skeletal muscle. II. Immunoreactivity in patients with Xp21 muscular dystrophy","volume":"94","author":"Nicholson","year":"1989","journal-title":"J. Neurol. Sci."},{"key":"2023072902454776000_Nigroetal1996","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1038\/ng1096-195","article-title":"Autosomal recessive limb-girdle muscular dystrophy, LGMD2F, is caused by a mutation in the delta-sarcoglycan gene","volume":"14","author":"Nigro","year":"1996","journal-title":"Nat. Genet."},{"key":"2023072902454776000_Noguchietal1995","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1126\/science.270.5237.819","article-title":"Mutations in the dystrophin-associated protein gamma-sarcoglycan in chromosome 13 muscular dystrophy","volume":"270","author":"Noguchi","year":"1995","journal-title":"Science"},{"key":"2023072902454776000_Pimorady-Esfahanietal1997","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1002\/(SICI)1097-4598(199702)20:2<158::AID-MUS4>3.0.CO;2-B","article-title":"Macrophages and dendritic cells in normal and regenerating murine skeletal muscle","volume":"20","author":"Pimorady-Esfahani","year":"1997","journal-title":"Muscle Nerve"},{"key":"2023072902454776000_Roberdsetal1993","doi-asserted-by":"crossref","first-page":"23739","DOI":"10.1016\/S0021-9258(20)80440-2","article-title":"Primary structure and muscle-specific expression of the 50-kDa dystrophin-associated glycoprotein (adhalin)","volume":"268","author":"Roberds","year":"1993","journal-title":"J. Biol. Chem."},{"key":"2023072902454776000_RoddieandBundey1992","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1136\/jmg.29.8.555","article-title":"Racial distribution of Duchenne muscular dystrophy in the West Midlands region of Britain","volume":"29","author":"Roddie","year":"1992","journal-title":"J. Med. Genet."},{"key":"2023072902454776000_Rybakovaetal2000","doi-asserted-by":"crossref","first-page":"1209","DOI":"10.1083\/jcb.150.5.1209","article-title":"The dystrophin complex forms a mechanically strong link between the sarcolemma and costameric actin","volume":"150","author":"Rybakova","year":"2000","journal-title":"J. Cell Biol."},{"key":"2023072902454776000_Suekietal1993","first-page":"160","article-title":"Novel interactions between dermal dendrocytes and mast cells in human skin. Implications for hemostasis and matrix repair","volume":"69","author":"Sueki","year":"1993","journal-title":"Lab. Invest."},{"key":"2023072902454776000_Tezaketal2000"},{"key":"2023072902454776000_Thomasetal1998","doi-asserted-by":"crossref","first-page":"15090","DOI":"10.1073\/pnas.95.25.15090","article-title":"Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle","volume":"95","author":"Thomas","year":"1998","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2023072902454776000_Tinsleyetal1998","doi-asserted-by":"crossref","first-page":"1441","DOI":"10.1038\/4033","article-title":"Expression of full-length utrophin prevents muscular dystrophy in mdx mice","volume":"4","author":"Tinsley","year":"1998","journal-title":"Nat. Med."},{"key":"2023072902454776000_Toyofukoetal1992","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1002\/aja.1001930406","article-title":"Expression of \u03b1-cardiac and \u03b1-skeletal actin mRNAs in relation to innervation in regenerating and non-regenerating rat skeletal muscles","volume":"193","author":"Toyofuko","year":"1992","journal-title":"Dev. Dyn."},{"key":"2023072902454776000_Whalenetal1979","doi-asserted-by":"crossref","first-page":"5197","DOI":"10.1073\/pnas.76.10.5197","article-title":"Contractile protein isozymes in muscle developmentidentification of an embryonic form of myosin heavy chain","volume":"76","author":"Whalen","year":"1979","journal-title":"Proc. Nat. Acad. Sci. 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