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Spencer, Melissa J

Title(s)Professor, Neurology
Phone3107945225
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    Collapse Research 
    Collapse Research Activities and Funding
    FASEB SRC on Calpains in Health and Disease
    NIH/NINDS R13NS111915Jul 1, 2019 - Jun 30, 2020
    Role: Principal Investigator
    Identification of Enhancers of Therapeutic Exon Skipping for DMD
    NIH/NIAMS RC1AR058333Sep 25, 2009 - Aug 31, 2011
    Role: Co-Principal Investigator
    UCLA Muscular Dystrophy Core Center
    NIH/NIAMS P30AR057230Apr 1, 2009 - Mar 31, 2019
    Role: Principal Investigator
    Pathogenic Mechanisms in Limb Girdle Muscular Dystrophies
    NIH/NIAMS R01AR052693Aug 10, 2007 - Jul 31, 2013
    Role: Principal Investigator
    FASEB Summer Conference on: The Biology of Calpains in Health and Disease
    NIH/NIAMS R13AR055422Jul 1, 2007 - Jun 30, 2008
    Role: Principal Investigator
    Mechanisms Underlying Limb Girdle Muscular Dystrophy2A Due to Calpain 3 Mutations
    NIH/NIAMS R01AR048177Sep 24, 2001 - Aug 31, 2017
    Role: Principal Investigator
    THERAPEUTIC APPROACHES FOR MUSCULAR DYSTROPHY
    NIH/NIAMS R01AR046911May 5, 2000 - Apr 30, 2008
    Role: Principal Investigator
    CALPAIN REGULATION OF MUSCLE NECROSIS AND REGENERATION
    NIH/NIAMS R03AR045838Sep 30, 1998 - Aug 31, 2001
    Role: Principal Investigator
    CYTOTOXIC T CELLS AND MDX DYSTROPHY
    NIH/NIAMS F32AR008439Dec 20, 1996
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
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    1. Kramerova I, Kumagai-Cresse C, Ermolova N, Mokhonova E, Marinov M, Capote J, Becerra D, Quattrocelli M, Crosbie RH, Welch E, McNally EM, Spencer MJ. Spp1 (osteopontin) promotes TGFß processing in fibroblasts of dystrophin deficient muscles through matrix metalloproteinases. Hum Mol Genet. 2019 Aug 14. PMID: 31411676.
      View in: PubMed
    2. Malik R, Meng H, Wongkongkathep P, Corrales CI, Sepanj N, Atlasi RS, Klärner FG, Schrader T, Spencer MJ, Loo JA, Wiedau M, Bitan G. The molecular tweezer CLR01 inhibits aberrant superoxide dismutase 1 (SOD1) self-assembly in vitro and in the G93A-SOD1 mouse model of ALS. J Biol Chem. 2019 03 08; 294(10):3501-3513. PMID: 30602569.
      View in: PubMed
    3. Ji Y, Liu X, Huang M, Jiang J, Liao YP, Liu Q, Chang CH, Liao H, Lu J, Wang X, Spencer MJ, Meng H. Development of self-assembled multi-arm polyrotaxanes nanocarriers for systemic plasmid delivery in vivo. Biomaterials. 2019 02; 192:416-428. PMID: 30500723.
      View in: PubMed
    4. Kramerova I, Torres JA, Eskin A, Nelson SF, Spencer MJ. Calpain 3 and CaMKIIß signaling are required to induce HSP70 necessary for adaptive muscle growth after atrophy. Hum Mol Genet. 2018 05 01; 27(9):1642-1653. PMID: 29528394.
      View in: PubMed
    5. Wang DW, Mokhonova EI, Kendall GC, Becerra D, Naeini YB, Cantor RM, Spencer MJ, Nelson SF, Miceli MC. Repurposing Dantrolene for Long-Term Combination Therapy to Potentiate Antisense-Mediated DMD Exon Skipping in the mdx Mouse. Mol Ther Nucleic Acids. 2018 Jun 01; 11:180-191. PMID: 29858053.
      View in: PubMed
    6. Hicks MR, Hiserodt J, Paras K, Fujiwara W, Eskin A, Jan M, Xi H, Young CS, Evseenko D, Nelson SF, Spencer MJ, Handel BV, Pyle AD. ERBB3 and NGFR mark a distinct skeletal muscle progenitor cell in human development and hPSCs. Nat Cell Biol. 2018 01; 20(1):46-57. PMID: 29255171.
      View in: PubMed
    7. Quattrocelli M, Capote J, Ohiri JC, Warner JL, Vo AH, Earley JU, Hadhazy M, Demonbreun AR, Spencer MJ, McNally EM. Genetic modifiers of muscular dystrophy act on sarcolemmal resealing and recovery from injury. PLoS Genet. 2017 Oct; 13(10):e1007070. PMID: 29065150.
      View in: PubMed
    8. Yao J, Guihard PJ, Wu X, Blazquez-Medela AM, Spencer MJ, Jumabay M, Tontonoz P, Fogelman AM, Boström KI, Yao Y. Vascular endothelium plays a key role in directing pulmonary epithelial cell differentiation. J Cell Biol. 2017 10 02; 216(10):3369-3385. PMID: 28838957.
      View in: PubMed
    9. Young CS, Mokhonova E, Quinonez M, Pyle AD, Spencer MJ. Creation of a Novel Humanized Dystrophic Mouse Model of Duchenne Muscular Dystrophy and Application of a CRISPR/Cas9 Gene Editing Therapy. J Neuromuscul Dis. 2017; 4(2):139-145. PMID: 28505980.
      View in: PubMed
    10. Quattrocelli M, Spencer MJ, McNally EM. Outside in: The matrix as a modifier of muscular dystrophy. Biochim Biophys Acta Mol Cell Res. 2017 Mar; 1864(3):572-579. PMID: 28011285.
      View in: PubMed
    11. Gibbs EM, Marshall JL, Ma E, Nguyen TM, Hong G, Lam JS, Spencer MJ, Crosbie-Watson RH. High levels of sarcospan are well tolerated and act as a sarcolemmal stabilizer to address skeletal muscle and pulmonary dysfunction in DMD. Hum Mol Genet. 2016 12 15; 25(24):5395-5406. PMID: 27798107.
      View in: PubMed
    12. Capote J, Kramerova I, Martinez L, Vetrone S, Barton ER, Sweeney HL, Miceli MC, Spencer MJ. Osteopontin ablation ameliorates muscular dystrophy by shifting macrophages to a pro-regenerative phenotype. J Cell Biol. 2016 04 25; 213(2):275-88. PMID: 27091452.
      View in: PubMed
    13. Kramerova I, Ermolova N, Eskin A, Hevener A, Quehenberger O, Armando AM, Haller R, Romain N, Nelson SF, Spencer MJ. Failure to up-regulate transcription of genes necessary for muscle adaptation underlies limb girdle muscular dystrophy 2A (calpainopathy). Hum Mol Genet. 2016 06 01; 25(11):2194-2207. PMID: 27005420.
      View in: PubMed
    14. DiFranco M, Kramerova I, Vergara JL, Spencer MJ. Attenuated Ca(2+) release in a mouse model of limb girdle muscular dystrophy 2A. Skelet Muscle. 2016; 6:11. PMID: 26913171.
      View in: PubMed
    15. Young CS, Hicks MR, Ermolova NV, Nakano H, Jan M, Younesi S, Karumbayaram S, Kumagai-Cresse C, Wang D, Zack JA, Kohn DB, Nakano A, Nelson SF, Miceli MC, Spencer MJ, Pyle AD. A Single CRISPR-Cas9 Deletion Strategy that Targets the Majority of DMD Patients Restores Dystrophin Function in hiPSC-Derived Muscle Cells. Cell Stem Cell. 2016 Apr 07; 18(4):533-40. PMID: 26877224.
      View in: PubMed
    16. Martinez L, Ermolova NV, Ishikawa TO, Stout DB, Herschman HR, Spencer MJ. A reporter mouse for optical imaging of inflammation in mdx muscles. Skelet Muscle. 2015; 5:15. PMID: 25949789.
      View in: PubMed
    17. Mokhonova EI, Avliyakulov NK, Kramerova I, Kudryashova E, Haykinson MJ, Spencer MJ. The E3 ubiquitin ligase TRIM32 regulates myoblast proliferation by controlling turnover of NDRG2. Hum Mol Genet. 2015 May 15; 24(10):2873-83. PMID: 25701873.
      View in: PubMed
    18. Ermolova N, Kramerova I, Spencer MJ. Autolytic activation of calpain 3 proteinase is facilitated by calmodulin protein. J Biol Chem. 2015 Jan 09; 290(2):996-1004. PMID: 25389288.
      View in: PubMed
    19. Villalta SA, Rosenthal W, Martinez L, Kaur A, Sparwasser T, Tidball JG, Margeta M, Spencer MJ, Bluestone JA. Regulatory T cells suppress muscle inflammation and injury in muscular dystrophy. Sci Transl Med. 2014 Oct 15; 6(258):258ra142. PMID: 25320234.
      View in: PubMed
    20. Kendall GC, Mokhonova EI, Moran M, Sejbuk NE, Wang DW, Silva O, Wang RT, Martinez L, Lu QL, Damoiseaux R, Spencer MJ, Nelson SF, Miceli MC. Dantrolene enhances antisense-mediated exon skipping in human and mouse models of Duchenne muscular dystrophy. Sci Transl Med. 2012 Dec 12; 4(164):164ra160. PMID: 23241744.
      View in: PubMed
    21. Chao LC, Wroblewski K, Ilkayeva OR, Stevens RD, Bain J, Meyer GA, Schenk S, Martinez L, Vergnes L, Narkar VA, Drew BG, Hong C, Boyadjian R, Hevener AL, Evans RM, Reue K, Spencer MJ, Newgard CB, Tontonoz P. Skeletal muscle Nur77 expression enhances oxidative metabolism and substrate utilization. J Lipid Res. 2012 Dec; 53(12):2610-9. PMID: 23028113.
      View in: PubMed
    22. Jaka O, Kramerova I, Azpitarte M, López de Munain A, Spencer M, Sáenz A. C3KO mouse expression analysis: downregulation of the muscular dystrophy Ky protein and alterations in muscle aging. Neurogenetics. 2012 Nov; 13(4):347-57. PMID: 22820870.
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    23. Kudryashova E, Kramerova I, Spencer MJ. Satellite cell senescence underlies myopathy in a mouse model of limb-girdle muscular dystrophy 2H. J Clin Invest. 2012 May; 122(5):1764-76. PMID: 22505452.
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    24. Kudryashova E, Struyk A, Mokhonova E, Cannon SC, Spencer MJ. The common missense mutation D489N in TRIM32 causing limb girdle muscular dystrophy 2H leads to loss of the mutated protein in knock-in mice resulting in a Trim32-null phenotype. Hum Mol Genet. 2011 Oct 15; 20(20):3925-32. PMID: 21775502.
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    25. Ermolova N, Kudryashova E, DiFranco M, Vergara J, Kramerova I, Spencer MJ. Pathogenity of some limb girdle muscular dystrophy mutations can result from reduced anchorage to myofibrils and altered stability of calpain 3. Hum Mol Genet. 2011 Sep 01; 20(17):3331-45. PMID: 21624972.
      View in: PubMed
    26. Kim MH, Kay DI, Rudra RT, Chen BM, Hsu N, Izumiya Y, Martinez L, Spencer MJ, Walsh K, Grinnell AD, Crosbie RH. Myogenic Akt signaling attenuates muscular degeneration, promotes myofiber regeneration and improves muscle function in dystrophin-deficient mdx mice. Hum Mol Genet. 2011 Apr 01; 20(7):1324-38. PMID: 21245083.
      View in: PubMed
    27. Shieh PB, Kudryashova E, Spencer MJ. Limb-girdle muscular dystrophy 2H and the role of TRIM32. Handb Clin Neurol. 2011; 101:125-33. PMID: 21496629.
      View in: PubMed
    28. Nelson SF, Crosbie RH, Miceli MC, Spencer MJ. Emerging genetic therapies to treat Duchenne muscular dystrophy. Curr Opin Neurol. 2009 Oct; 22(5):532-8. PMID: 19745732.
      View in: PubMed
    29. Mellgren RL, Miyake K, Kramerova I, Spencer MJ, Bourg N, Bartoli M, Richard I, Greer PA, McNeil PL. Calcium-dependent plasma membrane repair requires m- or mu-calpain, but not calpain-3, the proteasome, or caspases. Biochim Biophys Acta. 2009 Dec; 1793(12):1886-93. PMID: 19781581.
      View in: PubMed
    30. Kramerova I, Kudryashova E, Wu B, Germain S, Vandenborne K, Romain N, Haller RG, Verity MA, Spencer MJ. Mitochondrial abnormalities, energy deficit and oxidative stress are features of calpain 3 deficiency in skeletal muscle. Hum Mol Genet. 2009 Sep 01; 18(17):3194-205. PMID: 19483197.
      View in: PubMed
    31. Vetrone SA, Montecino-Rodriguez E, Kudryashova E, Kramerova I, Hoffman EP, Liu SD, Miceli MC, Spencer MJ. Osteopontin promotes fibrosis in dystrophic mouse muscle by modulating immune cell subsets and intramuscular TGF-beta. J Clin Invest. 2009 Jun; 119(6):1583-94. PMID: 19451692.
      View in: PubMed
    32. Kudryashova E, Wu J, Havton LA, Spencer MJ. Deficiency of the E3 ubiquitin ligase TRIM32 in mice leads to a myopathy with a neurogenic component. Hum Mol Genet. 2009 Apr 01; 18(7):1353-67. PMID: 19155210.
      View in: PubMed
    33. Beckmann JS, Spencer M. Calpain 3, the "gatekeeper" of proper sarcomere assembly, turnover and maintenance. Neuromuscul Disord. 2008 Dec; 18(12):913-21. PMID: 18974005.
      View in: PubMed
    34. Kramerova I, Kudryashova E, Wu B, Ottenheijm C, Granzier H, Spencer MJ. Novel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscle. Hum Mol Genet. 2008 Nov 01; 17(21):3271-80. PMID: 18676612.
      View in: PubMed
    35. Groshong JS, Spencer MJ, Bhattacharyya BJ, Kudryashova E, Vohra BP, Zayas R, Wollmann RL, Miller RJ, Gomez CM. Calpain activation impairs neuromuscular transmission in a mouse model of the slow-channel myasthenic syndrome. J Clin Invest. 2007 Oct; 117(10):2903-12. PMID: 17853947.
      View in: PubMed
    36. Cohen N, Kudryashova E, Kramerova I, Anderson LV, Beckmann JS, Bushby K, Spencer MJ. Identification of putative in vivo substrates of calpain 3 by comparative proteomics of overexpressing transgenic and nontransgenic mice. Proteomics. 2006 Nov; 6(22):6075-84. PMID: 17051641.
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    37. Kramerova I, Kudryashova E, Wu B, Spencer MJ. Regulation of the M-cadherin-beta-catenin complex by calpain 3 during terminal stages of myogenic differentiation. Mol Cell Biol. 2006 Nov; 26(22):8437-47. PMID: 16982691.
      View in: PubMed
    38. Kramerova I, Beckmann JS, Spencer MJ. Molecular and cellular basis of calpainopathy (limb girdle muscular dystrophy type 2A). Biochim Biophys Acta. 2007 Feb; 1772(2):128-44. PMID: 16934440.
      View in: PubMed
    39. Nazarian R, Starcevic M, Spencer MJ, Dell'Angelica EC. Reinvestigation of the dysbindin subunit of BLOC-1 (biogenesis of lysosome-related organelles complex-1) as a dystrobrevin-binding protein. Biochem J. 2006 May 01; 395(3):587-98. PMID: 16448387.
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    40. Kudryashova E, Kudryashov D, Kramerova I, Spencer MJ. Trim32 is a ubiquitin ligase mutated in limb girdle muscular dystrophy type 2H that binds to skeletal muscle myosin and ubiquitinates actin. J Mol Biol. 2005 Nov 25; 354(2):413-24. PMID: 16243356.
      View in: PubMed
    41. Huebsch KA, Kudryashova E, Wooley CM, Sher RB, Seburn KL, Spencer MJ, Cox GA. Mdm muscular dystrophy: interactions with calpain 3 and a novel functional role for titin's N2A domain. Hum Mol Genet. 2005 Oct 01; 14(19):2801-11. PMID: 16115818.
      View in: PubMed
    42. Kramerova I, Kudryashova E, Venkatraman G, Spencer MJ. Calpain 3 participates in sarcomere remodeling by acting upstream of the ubiquitin-proteasome pathway. Hum Mol Genet. 2005 Aug 01; 14(15):2125-34. PMID: 15961411.
      View in: PubMed
    43. Kramerova I, Kudryashova E, Tidball JG, Spencer MJ. Null mutation of calpain 3 (p94) in mice causes abnormal sarcomere formation in vivo and in vitro. Hum Mol Genet. 2004 Jul 01; 13(13):1373-88. PMID: 15138196.
      View in: PubMed
    44. Guyon JR, Kudryashova E, Potts A, Dalkilic I, Brosius MA, Thompson TG, Beckmann JS, Kunkel LM, Spencer MJ. Calpain 3 cleaves filamin C and regulates its ability to interact with gamma- and delta-sarcoglycans. Muscle Nerve. 2003 Oct; 28(4):472-83. PMID: 14506720.
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    45. Gosselin LE, Barkley JE, Spencer MJ, McCormick KM, Farkas GA. Ventilatory dysfunction in mdx mice: impact of tumor necrosis factor-alpha deletion. Muscle Nerve. 2003 Sep; 28(3):336-43. PMID: 12929194.
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    46. Tidball JG, Spencer MJ. Skipping to new gene therapies for muscular dystrophy. Nat Med. 2003 Aug; 9(8):997-8. PMID: 12894160.
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    47. Hodgetts SI, Spencer MJ, Grounds MD. A role for natural killer cells in the rapid death of cultured donor myoblasts after transplantation. Transplantation. 2003 Mar 27; 75(6):863-71. PMID: 12660516.
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    48. Tidball JG, Spencer MJ. Expression of a calpastatin transgene slows muscle wasting and obviates changes in myosin isoform expression during murine muscle disuse. J Physiol. 2002 12 15; 545(3):819-28. PMID: 12482888.
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    49. Spencer MJ, Mellgren RL. Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology. Hum Mol Genet. 2002 Oct 01; 11(21):2645-55. PMID: 12354790.
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    50. Spencer MJ, Guyon JR, Sorimachi H, Potts A, Richard I, Herasse M, Chamberlain J, Dalkilic I, Kunkel LM, Beckmann JS. Stable expression of calpain 3 from a muscle transgene in vivo: immature muscle in transgenic mice suggests a role for calpain 3 in muscle maturation. Proc Natl Acad Sci U S A. 2002 Jun 25; 99(13):8874-9. PMID: 12084932.
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    51. Taveau M, Stockholm D, Spencer M, Richard I. Quantification of splice variants using molecular beacon or scorpion primers. Anal Biochem. 2002 Jun 15; 305(2):227-35. PMID: 12054451.
      View in: PubMed
    52. Spencer MJ, Tidball JG. Do immune cells promote the pathology of dystrophin-deficient myopathies? Neuromuscul Disord. 2001 Sep; 11(6-7):556-64. PMID: 11525885.
      View in: PubMed
    53. Spencer MJ, Marino MW, Winckler WM. Altered pathological progression of diaphragm and quadriceps muscle in TNF-deficient, dystrophin-deficient mice. Neuromuscul Disord. 2000 Dec; 10(8):612-9. PMID: 11053690.
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