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Article

Brown, S C and Winder, S J and on behalf of the ENMC DGpathy Study Group (2017) The 220th ENMC workshop: Dystroglycan and the Dystroglycanopathies held on the 27-29 May 2016, Naarden, The Netherlands. Neuromuscular Disorders, 27 (4). pp. 387-395.

Booler, H and Pagalday-Vergara, V and Williams, J L and Hopkinson, M and Brown, S C (2017) Evidence of early defects in Cajal-Retzius cell localization during brain development in a mouse model of dystroglycanopathy. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 43 (4). pp. 330-345.

Hildyard, J C W and Lacey, E and Booler, H and Hopkinson, M and Wells, D J and Brown, S C (2016) Transgenic Rescue of the LARGEmyd Mouse: A LARGE Therapeutic Window? PLoS One, 11 (7). e0159853.

Booler, H and Williams, J and Hopkinson, M and Brown, S C (2016) Degree of Cajal-Retzius cell mislocalisation correlates with the severity of structural brain defects in mouse models of dystroglycanopathy. Brain Pathology, 26 (4). pp. 465-478.

Kim, J and Hopkinson, M and Kavishwar, M and Fernandez-Fuente, M and Brown, S C (2016) Prenatal muscle development in a mouse model for the secondary dystroglycanopathies. Skeletal Muscle, 6 (3).

Humphrey, E L and Lacey, E and Le, L T and Feng, L and Sciandra, F and Morris, C R and Hewitt, J E and Holt, I and Brancaccio, A and Barresi, R and Sewry, C A and Brown, S C and Morris, G E (2015) A new monoclonal antibody DAG-6F4 against human alpha-dystroglycan reveals reduced core protein in some, but not all, dystroglycanopathy patients. Neuromuscular Disorders, 25 (1). pp. 32-42.

Fernandez-Fuente, M and Terracciano, C M and Martin-Duque, P and Brown, S C and Vassaux, G and Piercy, R J (2014) Calcium Homeostasis in Myogenic Differentiation Factor 1 (MyoD)-Transformed, Virally-Transduced, Skin-Derived Equine Myotubes. PLoS One, 9 (8). e105971.

Fernandez-Fuente, M and Martin-Duque, P and Vassaux, G and Brown, S C and Muntoni, F and Terracciano, C M and Piercy, R J (2014) Adenovirus-mediated expression of Myogenic Differentiation Factor 1 (MyoD) in equine and human dermal fibroblasts enables their conversion to caffeine-sensitive myotubes. Neuromuscular Disorders, 24 (3). pp. 250-258.

Humphrey, E L and Lacey, E and Le, L T and Sciandra, F and Morris, C and Hewitt, J E and Holt, I and Brancaccio, A and Barresi, R and Sewry, C A and Brown, S C and Morris, G E (2014) A NEW MONOCLONAL ANTIBODY AGAINST HUMAN ALPHA-DYSTROGLYCAN HAS POTENTIAL DIAGNOSTIC APPLICATIONS. Neuromuscular Disorders, 24 (9-10). p. 918.

Fernandez-Fuente, M and Kim, J and Wells, D J and Brown, S C (2013) ASSESSMENT OF NEUROMUSCULAR JUNCTION ABNORMALITIES INDUCED BY ALPHA-DYSTROGLYCAN GLYCOSYLATION DEFECTS. Neuromuscular Disorders, 23 (9-10). pp. 781-782.

Kim, J and Upadhyaya, A and Fuente-Fernandez, M F and Whitmore, C and Brown, S C (2012) BASEMENT MEMBRANE DEPOSITION IN THE SKELETAL MUSCLE OF THE FKRPKNOCK-DOWN MOUSE. Neuromuscular Disorders, 22. S12-S13.

Ross, J and Benn, A and Jonuschies, J and Boldrin, L and Muntoni, F and Hewitt, J E and Brown, S C and Morgan, J E (2012) Defects in Glycosylation Impair Satellite Stem Cell Function and Niche Composition in the Muscles of the Dystrophic Largemyd Mouse. Stem Cells, 30 (10). pp. 2330-2341.

Kim, J and Jimenez-Mallebrera, C and Foley, A R and Fernandez-Fuente, M and Brown, S C and Torelli, S and Feng, L and Sewry, C A and Muntoni, F (2012) Flow cytometry analysis: a quantitative method for collagen VI deficiency screening. Neuromuscular Disorders, 22 (2). pp. 139-48.

Miller, J and Brown, S C (2012) Sun, Surf and Science. Neuromuscular Disorders, 22 (3). pp. 292-296.

Waite, A and Brown, S C and Blake, D J (2012) The dystrophin-glycoprotein complex in brain development and disease. TRENDS IN NEUROSCIENCES, 35 (8). pp. 487-496.

Whitmore, C and Ackroyd, M R and Ashraf, A and Brown, S C (2011) DEPOSITION OF THE INNER LIMITING MEMBRANE IN THE EYE OF A MOUSE MODEL FOR MUSCLE EYE BRAIN DISEASE. Neuromuscular Disorders, 21 (9-10). p. 665.

Ackroyd, M R and Whitmore, C and Prior, S and Kalurachchi, M and Nikolic, M and Mayer, U and Muntoni, F and Brown, S C (2011) Fukutin-Related Protein Alters the Deposition of Laminin in the Eye and Brain. JOURNAL OF NEUROSCIENCE, 31 (36). pp. 12927-12935.

Whitmore, C and Ackroyd, M R and Ashraf, A and Alyoshkin, B and Muntoni, F and Brown, S C (2011) GENERATION OF A NEW MOUSE MODEL FOR THERAPEUTIC TESTING IN THE DYSTROGLYCANOPATHIES. Neuromuscular Disorders, 21 (9-10). p. 664.

Muntoni, F and Torelli, S and Wells, D J and Brown, S C (2011) Muscular dystrophies due to glycosylation defects: diagnosis and therapeutic strategies. Current Opinion in Neurology, 24 (5). pp. 437-442.

Chan, Y M and Brown, S C and Lu, Q (2011) Second International Workshop for Glycosylation Defects in Muscular Dystrophies, 11-12 November, 2010, Charlotte, USA. BRAIN PATHOLOGY, 21 (6). pp. 699-704.

Brockington, M A and Torelli, S and Sharp, P S and Liu, K and Cirak, S and Brown, S C and Wells, D J and Muntoni, F (2010) Transgenic Overexpression of LARGE Induces alpha-Dystroglycan Hyperglycosylation in Skeletal and Cardiac Muscle. PLoS One, 5 (12). e14434.

Matsakas, A and Otto, A and Elashry, M I and Brown, S C and Patel, K (2010) Altered primary and secondary myogenesis in the myostatin-null mouse. Rejuvenation Research, 13 (6). pp. 712-27.

Arechavala-Gomeza, V and Kinali, M and Feng, L and Brown, S C and Sewry, C and Morgan, J E and Muntoni, F (2010) Immunohistological intensity measurements as a tool to assess sarcolemma-associated protein expression. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY, 36 (4). pp. 265-74.

Ackroyd, M R and Skordis, L and Kaluarachchi, M and Godwin, J and Prior, S and Fidanboylu, M and Piercy, R J and Muntoni, F and Brown, S C (2009) Reduced expression of fukutin related protein in mice results in a model for fukutin related protein associated muscular dystrophies. BRAIN, 132. pp. 439-451.

Jimenez-Mallebrera, C and Torelli, S and Feng, L and Kim, J and Godfrey, C and Clement, E and Mein, R and Abbs, S and Brown, S C and Campbell, K P and Kröger, S and Talim, B and Topaloglu, H and Quinlivan, R and Roper, H and Childs, A M and Kinali, M and Sewry, C A and Muntoni, F (2009) A comparative study of alpha-dystroglycan glycosylation in dystroglycanopathies suggests that the hypoglycosylation of alpha-dystroglycan does not consistently correlate with clinical severity. Brain Pathology, 19 (4). pp. 596-611.

Fernandez-Fuente, M and Ames, E G and Wagner, M L and Zhou, H and Strom, M and Zammit, P S and Mickelson, J R and Muntoni, F and Brown, S C and Piercy, R J (2008) Assessment of the transformation of equine skin-derived fibroblasts to multinucleated skeletal myotubes following lentiviral-induced expression of equine myogenic differentiation 1. AMERICAN JOURNAL OF VETERINARY RESEARCH, 69 (12). pp. 1637-1645.

Brown, S C and Piercy, R J and Muntoni, F and Sewry, C A (2008) Investigating the pathology of Emery-Dreifuss muscular dystrophy. BIOCHEMICAL SOCIETY TRANSACTIONS, 36 (Pt 6). pp. 1335-1338.

Clement, E M and Godfrey, C and Tan, J and Brockington, M A and Torelli, S and Feng, L and Brown, S C and Jimenez-Mallebrera, C and Sewry, C A and Longman, C and Mein, R and Abbs, S and Vajsar, J and Schachter, H and Muntoni, F (2008) Mild POMGnT1 mutations underlie a novel limb-girdle muscular dystrophy variant. Archives of Neurology, 65 (1). pp. 137-4.

Piercy, R J and Zhou, H Y and Feng, L and Pombo, A and Muntoni, F and Brown, S C (2007) Desmin immunolocalisation in autosomal dominant Emery-Dreifuss muscular dystrophy. Neuromuscular Disorders, 17 (4). pp. 297-305.

Amthor, H and Macharia, R and Navarrete, R and Schuelke, M and Brown, S C and Otto, A and Voit, T and Muntoni, F and Vrbova, G and Partridge, T and Zammit, P and Bunger, L and Patel, K (2007) Lack of myostatin results in excessive muscle growth but impaired force generation. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 104 (6). pp. 1835-1840.

Conference or Workshop Item

Rivas, E and Vissing, J and Scoto, M and Fernandez-Fuente, M and Voit, T and Muntoni, F and Brown, S C (2014) PHENOTYPIC HETEROGENEITY OF THE C.919T > A FKRP GENE MUTATION IN HUMANS AND A MOUSE MODEL. In: UNSPECIFIED.

Blaeser, A and Sparks, S and Brown, S C and Campbell, K and Lu, Q (2014) Third International Workshop for Glycosylation Defects in Muscular Dystrophies, 18-19 April 2013, Charlotte, USA. In: UNSPECIFIED.

Kim, J and Fernandez-Fuente, M and Kavishwar, M and Brown, S C (2013) BASEMENT MEMBRANE DEPOSITION DURING MUSCLE DEVELOPMENT IN THE FKRP DEFICIENT MOUSE. In: UNSPECIFIED.

Wilson, E and Lacey, E and Holt, I and Sciandra, F and Brancaccio, A and Hewitt, J E and Kobayashi, K and Toda, T and Sewry, C A and Brown, S C and Morris, G E (2013) IMMUNOSTAINING OF THE SARCOLEMMA WITH A NEW MONOCLONAL ANTIBODY AGAINST ALPHA-DYSTROGLYCAN CORE AND ITS RELEVANCE TO DIAGNOSIS. In: UNSPECIFIED.

Lacey, E and Gullen, E and Hopkinson, M and Palmer, D B and Brown, S C (2013) INSIGHTS INTO THE ROLE OF INFLAMMATION IN DYSTROGLYCANOPATHIES - ANALYSING THE EFFECT OF ALPHA DYSTROGLYCAN GLYCOSYLATION ON IMMUNE FUNCTION. In: UNSPECIFIED.

Fernandez-Fuente, M and Whitmore, C and Kim, J and Parr, C and Brown, S C (2013) PROGRESSIVE MUSCULAR DYSTROPHY IN A MOUSE MODEL OF FKRP DEFICIENCY. In: UNSPECIFIED.

Rutkowski, A and Bonnemann, C and Brown, S C and Thorsteinsdottir, S and Dominov, J and Ruegg, M A and Matter, M L and Guttridge, D and Crosbie-Watson, R H and Kardon, G and Nagaraju, K and Girgenrath, M and Burkin, D J (2013) REPORT ON THE MYOMATRIX CONFERENCE APRIL 22-24, 2012, UNIVERSITY OF NEVADA, RENO, NEVADA, USA. In: UNSPECIFIED.

Whitmore, C and Booler, H and Parr, C and Ackroyd, M R and Fernandez-Fuente, M and Muntoni, F and Wells, D and Brown, S C (2012) ASSESSING THE LONG TERM EXPRESSION OF LARGE AS A POTENTIAL THERAPY IN A MOUSE MODEL OF LGMD2I. In: UNSPECIFIED.

Kim, J and Upadhyaya, A and Fuente-Fernandez, M and Whitmore, C and Brown, S C (2012) BASEMENT MEMBRANE DEPOSITION IN THE SKELETAL MUSCLE OF THE FKRP KNOCK-DOWN MOUSE. In: UNSPECIFIED.

Booler, H and Whitmore, C and Fernandez-Fuente, M and Brown, S C (2012) A COMPARISON OF THE STRUCTURAL BRAIN DEFECTS IN DYSTROGLYCANOPATHY MOUSE MODELS. In: UNSPECIFIED.

Booler, H and Whitmore, C and Fuente, M F and Brown, S C (2012) STRUCTURAL BRAIN DEFECTS ASSOCIATED WITH A KNOCK-DOWN IN FUKUTIN RELATED PROTEIN IN THE MOUSE. In: UNSPECIFIED.

Whitmore, C and Ackroyd, M R and Muses, S and Ashraf, A and Muntoni, F and Brown, S C and Wells, D J (2011) ASSESSING THE THERAPEUTIC POTENTIAL OF LARGE IN A NEW MOUSE MODEL OF DYSTROGLYCANOPATHY. In: UNSPECIFIED.

Fernandez-Fuente, M and Terracciano, C M and Pilsworth, R and Muntoni, F and Brown, S C and Piercy, R J (2011) CHARACTERISATION OF SARCOPLASMIC RETICULUM CALCIUM RELEASE IN EQUINE AND HUMAN MYOTUBES DERIVED FROM MYOD-TRANSFORMED FIBROBLASTS. In: UNSPECIFIED.

Ackroyd, M R and Whitmore, C and Nikolic, M and Mayer, U and Muntoni, F and Brown, S C (2011) NOVEL INSIGHT IN MUSCLE AND BRAIN INVOLVEMENT IN DYSTROGLYCANOPATHIES. In: UNSPECIFIED.

Ackroyd, M R and Whitmore, C and Mayer, U and Brown, S C (2011) A REDUCTION IN THE EXPRESSION OF FUKUTIN-RELATED PROTEIN LEADS TO THE ALTERED DEPOSITION OF MULTIPLE LAMININ ALPHA CHAINS IN A MOUSE MODEL FOR MUSCLE EYE BRAIN DISEASE. In: UNSPECIFIED.

This list was generated on Fri Sep 24 07:42:56 2021 BST.