Melissa J Spencer

Title(s)Professor, Neurology
SchoolMedicine
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    Optimizing and validation of gene therapy vectors to treat limb girdle muscular dystophy
    NIH R01NS117912Aug 1, 2020 - Apr 30, 2025
    Role: Principal Investigator
    FASEB SRC on Calpains in Health and Disease
    NIH R13NS111915Jul 1, 2019 - Jun 30, 2020
    Role: Principal Investigator
    Identification of Enhancers of Therapeutic Exon Skipping for DMD
    NIH RC1AR058333Sep 25, 2009 - Aug 31, 2011
    Role: Co-Principal Investigator
    UCLA Muscular Dystrophy Core Center
    NIH P30AR057230Apr 1, 2009 - Mar 31, 2021
    Role: Principal Investigator
    Pathogenic Mechanisms in Limb Girdle Muscular Dystrophies
    NIH R01AR052693Aug 10, 2007 - Jul 31, 2013
    Role: Principal Investigator
    FASEB Summer Conference on: The Biology of Calpains in Health and Disease
    NIH R13AR055422Jul 1, 2007 - Jun 30, 2008
    Role: Principal Investigator
    Mechanisms Underlying Limb Girdle Muscular Dystrophy2A Due to Calpain 3 Mutations
    NIH R01AR048177Sep 24, 2001 - Aug 31, 2017
    Role: Principal Investigator
    THERAPEUTIC APPROACHES FOR MUSCULAR DYSTROPHY
    NIH R01AR046911May 5, 2000 - Apr 30, 2008
    Role: Principal Investigator
    CALPAIN REGULATION OF MUSCLE NECROSIS AND REGENERATION
    NIH R03AR045838Sep 30, 1998 - Aug 31, 2001
    Role: Principal Investigator
    CYTOTOXIC T CELLS AND MDX DYSTROPHY
    NIH F32AR008439Dec 20, 1996
    Role: Principal Investigator

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    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. to make corrections and additions.
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    Altmetrics Details PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Single cell and TCR analysis of immune cells from AAV gene therapy-dosed Duchenne muscular dystrophy patients. Mol Ther Methods Clin Dev. 2024 Dec 12; 32(4):101349. Emami MR, Brimble MA, Espinoza A, Owens J, Whiteley LO, Casinghino S, Lanz TA, Farahat PK, Pellegrini M, Young CS, Thomas PG, McNally EM, Villalta SA, Schattgen SA, Spencer MJ. PMID: 39524974; PMCID: PMC11546459.
      View in: PubMed   Mentions:
    2. Expert panel curation of 31 genes in relation to limb girdle muscular dystrophy. Ann Clin Transl Neurol. 2024 Sep; 11(9):2268-2276. Mohan S, McNulty S, Thaxton C, Elnagheeb M, Owens E, Flowers M, Nunnery T, Self A, Palus B, Gorokhova S, Kennedy A, Niu Z, Johari M, Maiga AB, Macalalad K, Clause AR, Beckmann JS, Bronicki L, Cooper ST, Ganesh VS, Kang PB, Kesari A, Lek M, Levy J, Rufibach L, Savarese M, Spencer MJ, Straub V, Tasca G, Weihl CC. PMID: 39215466; PMCID: PMC11537137.
      View in: PubMed   Mentions:    Fields:    Translation:Humans
    3. Expert Panel Curation of 31 Genes in Relation to Limb Girdle Muscular Dystrophy. bioRxiv. 2024 May 06. Mohan S, McNulty S, Thaxton C, Elnagheeb M, Owens E, Flowers M, Nunnery T, Self A, Palus B, Gorokhova S, Kennedy A, Niu Z, Johari M, Maiga AB, Macalalad K, Clause AR, Beckmann JS, Bronicki L, Cooper ST, Ganesh VS, Kang PB, Kesari A, Lek M, Levy J, Rufibach L, Savarese M, Spencer MJ, Straub V, Tasca G, Weihl CC. PMID: 38765987; PMCID: PMC11100593.
      View in: PubMed   Mentions:
    4. The super-healing MRL strain promotes muscle growth in muscular dystrophy through a regenerative extracellular matrix. JCI Insight. 2024 Jan 04; 9(3). O'Brien JG, Willis AB, Long AM, Kwon J, Lee G, Li FW, Page PG, Vo AH, Hadhazy M, Spencer MJ, Crosbie RH, Demonbreun AR, McNally EM. PMID: 38175727; PMCID: PMC11143963.
      View in: PubMed   Mentions: 2     Fields:    Translation:AnimalsCells
    5. Successes and challenges in clinical gene therapy. Gene Ther. 2023 11; 30(10-11):738-746. Kohn DB, Chen YY, Spencer MJ. PMID: 37935854; PMCID: PMC10678346.
      View in: PubMed   Mentions: 15     Fields:    Translation:HumansCells
    6. Nanoparticles systemically biodistribute to regenerating skeletal muscle in DMD. J Nanobiotechnology. 2023 Aug 29; 21(1):303. Hicks MR, Liu X, Young CS, Saleh K, Ji Y, Jiang J, Emami MR, Mokhonova E, Spencer MJ, Meng H, Pyle AD. PMID: 37641124; PMCID: PMC10463982.
      View in: PubMed   Mentions: 1     Fields:    Translation:Animals
    7. Single-cell and spatial transcriptomics identify a macrophage population associated with skeletal muscle fibrosis. Sci Adv. 2023 07 07; 9(27):eadd9984. Coulis G, Jaime D, Guerrero-Juarez C, Kastenschmidt JM, Farahat PK, Nguyen Q, Pervolarakis N, McLinden K, Thurlow L, Movahedi S, Hughes BS, Duarte J, Sorn A, Montoya E, Mozaffar I, Dragan M, Othy S, Joshi T, Hans CP, Kimonis V, MacLean AL, Nie Q, Wallace LM, Harper SQ, Mozaffar T, Hogarth MW, Bhattacharya S, Jaiswal JK, Golann DR, Su Q, Kessenbrock K, Stec M, Spencer MJ, Zamudio JR, Villalta SA. PMID: 37418531; PMCID: PMC10328414.
      View in: PubMed   Mentions: 24     Fields:    Translation:HumansAnimalsCells
    8. Innate and adaptive AAV-mediated immune responses in a mouse model of Duchenne muscular dystrophy. Mol Ther Methods Clin Dev. 2023 Sep 14; 30:90-102. Emami MR, Espinoza A, Young CS, Ma F, Farahat PK, Felgner PL, Chamberlain JS, Xu X, Pyle AD, Pellegrini M, Villalta SA, Spencer MJ. PMID: 37746243; PMCID: PMC10512012.
      View in: PubMed   Mentions: 4  
    9. Single-cell and spatial transcriptomics identify a macrophage population associated with skeletal muscle fibrosis. bioRxiv. 2023 Apr 18. Coulis G, Jaime D, Guerrero-Juarez C, Kastenschmidt JM, Farahat PK, Nguyen Q, Pervolarakis N, McLinden K, Thurlow L, Movahedi S, Duarte J, Sorn A, Montoya E, Mozaffar I, Dragan M, Othy S, Joshi T, Hans CP, Kimonis V, MacLean AL, Nie Q, Wallace LM, Harper SQ, Mozaffar T, Hogarth MW, Bhattacharya S, Jaiswal JK, Golann DR, Su Q, Kessenbrock K, Stec M, Spencer MJ, Zamudio JR, Villalta SA. PMID: 37131694; PMCID: PMC10153153.
      View in: PubMed   Mentions:
    10. A Phase II Trial of Guadecitabine in Children and Adults with SDH-Deficient GIST, Pheochromocytoma, Paraganglioma, and HLRCC-Associated Renal Cell Carcinoma. Clin Cancer Res. 2023 01 17; 29(2):341-348. Ligon JA, Sundby RT, Wedekind MF, Arnaldez FI, Del Rivero J, Wiener L, Srinivasan R, Spencer M, Carbonell A, Lei H, Shern J, Steinberg SM, Figg WD, Peer CJ, Zimmerman S, Moraly J, Xu X, Fox S, Chan K, Barbato MI, Andresson T, Taylor N, Pacak K, Killian JK, Dombi E, Linehan WM, Miettinen M, Piekarz R, Helman LJ, Meltzer P, Widemann B, Glod J. PMID: 36302175; PMCID: PMC9851965.
      View in: PubMed   Mentions: 6     Fields:    Translation:HumansCellsCTClinical Trials
    11. Single cell sequencing maps skeletal muscle cellular diversity as disease severity increases in dystrophic mouse models. iScience. 2022 Nov 18; 25(11):105415. Saleh KK, Xi H, Switzler C, Skuratovsky E, Romero MA, Chien P, Gibbs D, Gane L, Hicks MR, Spencer MJ, Pyle AD. PMID: 36388984; PMCID: PMC9646951.
      View in: PubMed   Mentions: 15  
    12. Molecular and cellular basis of genetically inherited skeletal muscle disorders. Nat Rev Mol Cell Biol. 2021 11; 22(11):713-732. Dowling JJ, Weihl CC, Spencer MJ. PMID: 34257452; PMCID: PMC9686310.
      View in: PubMed   Mentions: 42     Fields:    Translation:HumansCells
    13. A Small-Molecule Approach to Restore a Slow-Oxidative Phenotype and Defective CaMKIIβ Signaling in Limb Girdle Muscular Dystrophy. Cell Rep Med. 2020 10 20; 1(7):100122. Liu J, Campagna J, John V, Damoiseaux R, Mokhonova E, Becerra D, Meng H, McNally EM, Pyle AD, Kramerova I, Spencer MJ. PMID: 33205074; PMCID: PMC7659555.
      View in: PubMed   Mentions: 5     Fields:    Translation:AnimalsCells
    14. Genome Editing-Mediated Utrophin Upregulation in Duchenne Muscular Dystrophy Stem Cells. Mol Ther Nucleic Acids. 2020 Dec 04; 22:500-509. Sengupta K, Mishra MK, Loro E, Spencer MJ, Pyle AD, Khurana TS. PMID: 33230452; PMCID: PMC7554652.
      View in: PubMed   Mentions: 12  
    15. Myostatin inhibition promotes fast fibre hypertrophy but causes loss of AMP-activated protein kinase signalling and poor exercise tolerance in a model of limb-girdle muscular dystrophy R1/2A. J Physiol. 2020 09; 598(18):3927-3939. Kramerova I, Marinov M, Owens J, Lee SJ, Becerra D, Spencer MJ. PMID: 33460149.
      View in: PubMed   Mentions: 6     Fields:    Translation:HumansAnimals
    16. A Human Skeletal Muscle Atlas Identifies the Trajectories of Stem and Progenitor Cells across Development and from Human Pluripotent Stem Cells. Cell Stem Cell. 2020 Jul 02; 27(1):181-185. Xi H, Langerman J, Sabri S, Chien P, Young CS, Younesi S, Hicks M, Gonzalez K, Fujiwara W, Marzi J, Liebscher S, Spencer M, Van Handel B, Evseenko D, Schenke-Layland K, Plath K, Pyle AD. PMID: 32619514; PMCID: PMC9012334.
      View in: PubMed   Mentions: 25     Fields:    
    17. A Human Skeletal Muscle Atlas Identifies the Trajectories of Stem and Progenitor Cells across Development and from Human Pluripotent Stem Cells. Cell Stem Cell. 2020 07 02; 27(1):158-176.e10. Xi H, Langerman J, Sabri S, Chien P, Young CS, Younesi S, Hicks M, Gonzalez K, Fujiwara W, Marzi J, Liebscher S, Spencer M, Van Handel B, Evseenko D, Schenke-Layland K, Plath K, Pyle AD. PMID: 32396864; PMCID: PMC7367475.
      View in: PubMed   Mentions: 54     Fields:    Translation:HumansCells
    18. Spp1 (osteopontin) promotes TGFβ processing in fibroblasts of dystrophin-deficient muscles through matrix metalloproteinases. Hum Mol Genet. 2019 10 15; 28(20):3431-3442. 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. PMID: 31411676; PMCID: PMC7345878.
      View in: PubMed   Mentions: 43     Fields:    Translation:AnimalsCells
    19. CRISPR for Neuromuscular Disorders: Gene Editing and Beyond. Physiology (Bethesda). 2019 09 01; 34(5):341-353. Young CS, Pyle AD, Spencer MJ. PMID: 31389773; PMCID: PMC6863376.
      View in: PubMed   Mentions: 10     Fields:    Translation:HumansAnimalsCells
    20. 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. Malik R, Meng H, Wongkongkathep P, Corrales CI, Sepanj N, Atlasi RS, Klärner FG, Schrader T, Spencer MJ, Loo JA, Wiedau M, Bitan G. PMID: 30602569; PMCID: PMC6416427.
      View in: PubMed   Mentions: 20     Fields:    Translation:AnimalsCells
    21. Development of self-assembled multi-arm polyrotaxanes nanocarriers for systemic plasmid delivery in vivo. Biomaterials. 2019 02; 192:416-428. Ji Y, Liu X, Huang M, Jiang J, Liao YP, Liu Q, Chang CH, Liao H, Lu J, Wang X, Spencer MJ, Meng H. PMID: 30500723; PMCID: PMC6934403.
      View in: PubMed   Mentions: 9     Fields:    Translation:AnimalsCells
    22. Calpain 3 and CaMKIIβ signaling are required to induce HSP70 necessary for adaptive muscle growth after atrophy. Hum Mol Genet. 2018 05 01; 27(9):1642-1653. Kramerova I, Torres JA, Eskin A, Nelson SF, Spencer MJ. PMID: 29528394; PMCID: PMC5905633.
      View in: PubMed   Mentions: 9     Fields:    Translation:AnimalsCells
    23. 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. Wang DW, Mokhonova EI, Kendall GC, Becerra D, Naeini YB, Cantor RM, Spencer MJ, Nelson SF, Miceli MC. PMID: 29858053; PMCID: PMC5992346.
      View in: PubMed   Mentions: 7  
    24. "Of Mice and Measures": A Project to Improve How We Advance Duchenne Muscular Dystrophy Therapies to the Clinic. J Neuromuscul Dis. 2018; 5(4):407-417. Gordish-Dressman H, Willmann R, Dalle Pazze L, Kreibich A, van Putten M, Heydemann A, Bogdanik L, Lutz C, Davies K, Demonbreun AR, Duan D, Elsey D, Fukada SI, Girgenrath M, Patrick Gonzalez J, Grounds MD, Nichols A, Partridge T, Passini M, Sanarica F, Schnell FJ, Wells DJ, Yokota T, Young CS, Zhong Z, Spurney C, Spencer M, De Luca A, Nagaraju K, Aartsma-Rus A. PMID: 30198876; PMCID: PMC6218134.
      View in: PubMed   Mentions: 17     Fields:    Translation:Animals
    25. ERBB3 and NGFR mark a distinct skeletal muscle progenitor cell in human development and hPSCs. Nat Cell Biol. 2018 01; 20(1):46-57. 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. PMID: 29255171; PMCID: PMC5962356.
      View in: PubMed   Mentions: 109     Fields:    Translation:HumansCells
    26. Genetic modifiers of muscular dystrophy act on sarcolemmal resealing and recovery from injury. PLoS Genet. 2017 Oct; 13(10):e1007070. Quattrocelli M, Capote J, Ohiri JC, Warner JL, Vo AH, Earley JU, Hadhazy M, Demonbreun AR, Spencer MJ, McNally EM. PMID: 29065150; PMCID: PMC5669489.
      View in: PubMed   Mentions: 19     Fields:    Translation:AnimalsCells
    27. Vascular endothelium plays a key role in directing pulmonary epithelial cell differentiation. J Cell Biol. 2017 10 02; 216(10):3369-3385. Yao J, Guihard PJ, Wu X, Blazquez-Medela AM, Spencer MJ, Jumabay M, Tontonoz P, Fogelman AM, Boström KI, Yao Y. PMID: 28838957; PMCID: PMC5626536.
      View in: PubMed   Mentions: 20     Fields:    Translation:AnimalsCells
    28. 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. Young CS, Mokhonova E, Quinonez M, Pyle AD, Spencer MJ. PMID: 28505980; PMCID: PMC5565771.
      View in: PubMed   Mentions: 44     Fields:    Translation:HumansAnimalsCells
    29. Outside in: The matrix as a modifier of muscular dystrophy. Biochim Biophys Acta Mol Cell Res. 2017 Mar; 1864(3):572-579. Quattrocelli M, Spencer MJ, McNally EM. PMID: 28011285; PMCID: PMC5262521.
      View in: PubMed   Mentions: 16     Fields:    Translation:HumansAnimalsCells
    30. 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. Gibbs EM, Marshall JL, Ma E, Nguyen TM, Hong G, Lam JS, Spencer MJ, Crosbie-Watson RH. PMID: 27798107; PMCID: PMC5418831.
      View in: PubMed   Mentions: 17     Fields:    Translation:HumansAnimalsCells
    31. Osteopontin ablation ameliorates muscular dystrophy by shifting macrophages to a pro-regenerative phenotype. J Cell Biol. 2016 04 25; 213(2):275-88. Capote J, Kramerova I, Martinez L, Vetrone S, Barton ER, Sweeney HL, Miceli MC, Spencer MJ. PMID: 27091452; PMCID: PMC5084275.
      View in: PubMed   Mentions: 70     Fields:    Translation:AnimalsCells
    32. 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. Kramerova I, Ermolova N, Eskin A, Hevener A, Quehenberger O, Armando AM, Haller R, Romain N, Nelson SF, Spencer MJ. PMID: 27005420; PMCID: PMC5081050.
      View in: PubMed   Mentions: 18     Fields:    Translation:HumansAnimalsCells
    33. Attenuated Ca(2+) release in a mouse model of limb girdle muscular dystrophy 2A. Skelet Muscle. 2016; 6:11. DiFranco M, Kramerova I, Vergara JL, Spencer MJ. PMID: 26913171; PMCID: PMC4765215.
      View in: PubMed   Mentions: 12     Fields:    Translation:AnimalsCells
    34. 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. 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. PMID: 26877224; PMCID: PMC4826286.
      View in: PubMed   Mentions: 213     Fields:    Translation:HumansAnimalsCells
    35. A reporter mouse for optical imaging of inflammation in mdx muscles. Skelet Muscle. 2015; 5:15. Martinez L, Ermolova NV, Ishikawa TO, Stout DB, Herschman HR, Spencer MJ. PMID: 25949789; PMCID: PMC4422315.
      View in: PubMed   Mentions: 4     Fields:    
    36. The E3 ubiquitin ligase TRIM32 regulates myoblast proliferation by controlling turnover of NDRG2. Hum Mol Genet. 2015 May 15; 24(10):2873-83. Mokhonova EI, Avliyakulov NK, Kramerova I, Kudryashova E, Haykinson MJ, Spencer MJ. PMID: 25701873; PMCID: PMC4481581.
      View in: PubMed   Mentions: 28     Fields:    Translation:AnimalsCells
    37. Autolytic activation of calpain 3 proteinase is facilitated by calmodulin protein. J Biol Chem. 2015 Jan 09; 290(2):996-1004. Ermolova N, Kramerova I, Spencer MJ. PMID: 25389288; PMCID: PMC4294526.
      View in: PubMed   Mentions: 8     Fields:    Translation:HumansAnimalsCells
    38. Regulatory T cells suppress muscle inflammation and injury in muscular dystrophy. Sci Transl Med. 2014 Oct 15; 6(258):258ra142. Villalta SA, Rosenthal W, Martinez L, Kaur A, Sparwasser T, Tidball JG, Margeta M, Spencer MJ, Bluestone JA. PMID: 25320234; PMCID: PMC4889432.
      View in: PubMed   Mentions: 130     Fields:    Translation:HumansAnimalsCells
    39. Dantrolene enhances antisense-mediated exon skipping in human and mouse models of Duchenne muscular dystrophy. Sci Transl Med. 2012 Dec 12; 4(164):164ra160. 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. PMID: 23241744.
      View in: PubMed   Mentions: 53     Fields:    Translation:HumansAnimalsCells
    40. Skeletal muscle Nur77 expression enhances oxidative metabolism and substrate utilization. J Lipid Res. 2012 Dec; 53(12):2610-9. 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. PMID: 23028113; PMCID: PMC3494265.
      View in: PubMed   Mentions: 44     Fields:    Translation:AnimalsCells
    41. C3KO mouse expression analysis: downregulation of the muscular dystrophy Ky protein and alterations in muscle aging. Neurogenetics. 2012 Nov; 13(4):347-57. Jaka O, Kramerova I, Azpitarte M, López de Munain A, Spencer M, Sáenz A. PMID: 22820870.
      View in: PubMed   Mentions: 4     Fields:    Translation:HumansAnimals
    42. Satellite cell senescence underlies myopathy in a mouse model of limb-girdle muscular dystrophy 2H. J Clin Invest. 2012 May; 122(5):1764-76. Kudryashova E, Kramerova I, Spencer MJ. PMID: 22505452; PMCID: PMC3336976.
      View in: PubMed   Mentions: 61     Fields:    Translation:AnimalsCells
    43. 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. Kudryashova E, Struyk A, Mokhonova E, Cannon SC, Spencer MJ. PMID: 21775502; PMCID: PMC3177646.
      View in: PubMed   Mentions: 26     Fields:    Translation:Animals
    44. 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. Ermolova N, Kudryashova E, DiFranco M, Vergara J, Kramerova I, Spencer MJ. PMID: 21624972; PMCID: PMC3153300.
      View in: PubMed   Mentions: 23     Fields:    Translation:AnimalsCells
    45. 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. Kim MH, Kay DI, Rudra RT, Chen BM, Hsu N, Izumiya Y, Martinez L, Spencer MJ, Walsh K, Grinnell AD, Crosbie RH. PMID: 21245083; PMCID: PMC3049356.
      View in: PubMed   Mentions: 31     Fields:    Translation:HumansAnimalsCells
    46. Limb-girdle muscular dystrophy 2H and the role of TRIM32. Handb Clin Neurol. 2011; 101:125-33. Shieh PB, Kudryashova E, Spencer MJ. PMID: 21496629.
      View in: PubMed   Mentions: 24     Fields:    Translation:Humans
    47. Emerging genetic therapies to treat Duchenne muscular dystrophy. Curr Opin Neurol. 2009 Oct; 22(5):532-8. Nelson SF, Crosbie RH, Miceli MC, Spencer MJ. PMID: 19745732; PMCID: PMC2856442.
      View in: PubMed   Mentions: 26     Fields:    Translation:Humans
    48. 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. Mellgren RL, Miyake K, Kramerova I, Spencer MJ, Bourg N, Bartoli M, Richard I, Greer PA, McNeil PL. PMID: 19781581; PMCID: PMC2787696.
      View in: PubMed   Mentions: 40     Fields:    Translation:AnimalsCells
    49. 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. Kramerova I, Kudryashova E, Wu B, Germain S, Vandenborne K, Romain N, Haller RG, Verity MA, Spencer MJ. PMID: 19483197; PMCID: PMC2722983.
      View in: PubMed   Mentions: 33     Fields:    Translation:AnimalsCells
    50. 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. Vetrone SA, Montecino-Rodriguez E, Kudryashova E, Kramerova I, Hoffman EP, Liu SD, Miceli MC, Spencer MJ. PMID: 19451692; PMCID: PMC2689112.
      View in: PubMed   Mentions: 168     Fields:    Translation:HumansAnimalsCells
    51. 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. Kudryashova E, Wu J, Havton LA, Spencer MJ. PMID: 19155210; PMCID: PMC2722196.
      View in: PubMed   Mentions: 62     Fields:    Translation:AnimalsCells
    52. Calpain 3, the "gatekeeper" of proper sarcomere assembly, turnover and maintenance. Neuromuscul Disord. 2008 Dec; 18(12):913-21. Beckmann JS, Spencer M. PMID: 18974005; PMCID: PMC2614824.
      View in: PubMed   Mentions: 55     Fields:    Translation:HumansAnimalsCells
    53. 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. Kramerova I, Kudryashova E, Wu B, Ottenheijm C, Granzier H, Spencer MJ. PMID: 18676612; PMCID: PMC2566524.
      View in: PubMed   Mentions: 53     Fields:    Translation:HumansAnimalsCells
    54. Calpain activation impairs neuromuscular transmission in a mouse model of the slow-channel myasthenic syndrome. J Clin Invest. 2007 Oct; 117(10):2903-12. Groshong JS, Spencer MJ, Bhattacharyya BJ, Kudryashova E, Vohra BP, Zayas R, Wollmann RL, Miller RJ, Gomez CM. PMID: 17853947; PMCID: PMC1974862.
      View in: PubMed   Mentions: 20     Fields:    Translation:HumansAnimalsCells
    55. 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. Cohen N, Kudryashova E, Kramerova I, Anderson LV, Beckmann JS, Bushby K, Spencer MJ. PMID: 17051641.
      View in: PubMed   Mentions: 18     Fields:    Translation:Animals
    56. 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. Kramerova I, Kudryashova E, Wu B, Spencer MJ. PMID: 16982691; PMCID: PMC1636794.
      View in: PubMed   Mentions: 27     Fields:    Translation:AnimalsCells
    57. Molecular and cellular basis of calpainopathy (limb girdle muscular dystrophy type 2A). Biochim Biophys Acta. 2007 Feb; 1772(2):128-44. Kramerova I, Beckmann JS, Spencer MJ. PMID: 16934440.
      View in: PubMed   Mentions: 38     Fields:    Translation:HumansAnimals
    58. 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. Nazarian R, Starcevic M, Spencer MJ, Dell'Angelica EC. PMID: 16448387; PMCID: PMC1462696.
      View in: PubMed   Mentions: 38     Fields:    Translation:HumansAnimalsCells
    59. 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. Kudryashova E, Kudryashov D, Kramerova I, Spencer MJ. PMID: 16243356.
      View in: PubMed   Mentions: 92     Fields:    Translation:AnimalsCells
    60. 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. Huebsch KA, Kudryashova E, Wooley CM, Sher RB, Seburn KL, Spencer MJ, Cox GA. PMID: 16115818; PMCID: PMC1350399.
      View in: PubMed   Mentions: 23     Fields:    Translation:AnimalsCells
    61. Calpain 3 participates in sarcomere remodeling by acting upstream of the ubiquitin-proteasome pathway. Hum Mol Genet. 2005 Aug 01; 14(15):2125-34. Kramerova I, Kudryashova E, Venkatraman G, Spencer MJ. PMID: 15961411.
      View in: PubMed   Mentions: 61     Fields:    Translation:AnimalsCells
    62. 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. Kramerova I, Kudryashova E, Tidball JG, Spencer MJ. PMID: 15138196.
      View in: PubMed   Mentions: 80     Fields:    Translation:AnimalsCells
    63. Calpain 3 cleaves filamin C and regulates its ability to interact with gamma- and delta-sarcoglycans. Muscle Nerve. 2003 Oct; 28(4):472-83. Guyon JR, Kudryashova E, Potts A, Dalkilic I, Brosius MA, Thompson TG, Beckmann JS, Kunkel LM, Spencer MJ. PMID: 14506720.
      View in: PubMed   Mentions: 35     Fields:    Translation:AnimalsCells
    64. Ventilatory dysfunction in mdx mice: impact of tumor necrosis factor-alpha deletion. Muscle Nerve. 2003 Sep; 28(3):336-43. Gosselin LE, Barkley JE, Spencer MJ, McCormick KM, Farkas GA. PMID: 12929194.
      View in: PubMed   Mentions: 26     Fields:    Translation:AnimalsCells
    65. Skipping to new gene therapies for muscular dystrophy. Nat Med. 2003 Aug; 9(8):997-8. Tidball JG, Spencer MJ. PMID: 12894160.
      View in: PubMed   Mentions: 4     Fields:    Translation:HumansAnimalsCells
    66. A role for natural killer cells in the rapid death of cultured donor myoblasts after transplantation. Transplantation. 2003 Mar 27; 75(6):863-71. Hodgetts SI, Spencer MJ, Grounds MD. PMID: 12660516.
      View in: PubMed   Mentions: 5     Fields:    Translation:AnimalsCells
    67. 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. Tidball JG, Spencer MJ. PMID: 12482888; PMCID: PMC2290726.
      View in: PubMed   Mentions: 72     Fields:    Translation:HumansAnimalsCells
    68. Overexpression of a calpastatin transgene in mdx muscle reduces dystrophic pathology. Hum Mol Genet. 2002 Oct 01; 11(21):2645-55. Spencer MJ, Mellgren RL. PMID: 12354790.
      View in: PubMed   Mentions: 58     Fields:    Translation:AnimalsCells
    69. 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. Spencer MJ, Guyon JR, Sorimachi H, Potts A, Richard I, Herasse M, Chamberlain J, Dalkilic I, Kunkel LM, Beckmann JS. PMID: 12084932; PMCID: PMC124391.
      View in: PubMed   Mentions: 29     Fields:    Translation:HumansAnimalsCells
    70. Quantification of splice variants using molecular beacon or scorpion primers. Anal Biochem. 2002 Jun 15; 305(2):227-35. Taveau M, Stockholm D, Spencer M, Richard I. PMID: 12054451.
      View in: PubMed   Mentions: 3     Fields:    Translation:AnimalsCells
    71. Do immune cells promote the pathology of dystrophin-deficient myopathies? Neuromuscul Disord. 2001 Sep; 11(6-7):556-64. Spencer MJ, Tidball JG. PMID: 11525885.
      View in: PubMed   Mentions: 69     Fields:    Translation:HumansAnimalsCells
    72. Altered pathological progression of diaphragm and quadriceps muscle in TNF-deficient, dystrophin-deficient mice. Neuromuscul Disord. 2000 Dec; 10(8):612-9. Spencer MJ, Marino MW, Winckler WM. PMID: 11053690.
      View in: PubMed   Mentions: 19     Fields:    Translation:AnimalsCells
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