Molecular analysis of a spontaneous dystrophin `knockout' dog

S.J. Schatzberg; N.J. Olby; M. Breen; L.V.B. Anderson; C.F. Langford; H.F. Dickens; S.D. Wilton; C.J. Zeiss; M.M. Binns; J.N. Kornegay; G.E. Morris; N.J.H. Sharp

doi:10.1016/S0960-8966(99)00011-5

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Molecular analysis of a spontaneous dystrophin `knockout' dog

Journal article

Peer reviewed

Molecular analysis of a spontaneous dystrophin `knockout' dog

S.J. Schatzberg, N.J. Olby, M. Breen, L.V.B. Anderson, C.F. Langford, H.F. Dickens, S.D. Wilton, C.J. Zeiss, M.M. Binns, J.N. Kornegay, …

Neuromuscular Disorders, Vol.9(5), pp.289-295

1999

DOI: https://doi.org/10.1016/S0960-8966(99)00011-5

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Abstract

We have determined the molecular basis for skeletal myopathy and dilated cardiomyopathy in two male German short-haired pointer (GSHP) littermates. Analysis of skeletal muscle demonstrated a complete absence of dystrophin on Western blot analysis. PCR analysis of genomic DNA revealed a deletion encompassing the entire dystrophin gene. Molecular cytogenetic analysis of lymphocytes from the dam and both dystrophic pups confirmed a visible deletion in the p21 region of the affected canine X chromosome. Utrophin is up-regulated in the skeletal muscle, but does not appear to ameliorate the dystrophic canine phenotype. This new canine model should further our understanding of the physiological and biochemical processes in Duchenne muscular dystrophy.

Details

Title: Molecular analysis of a spontaneous dystrophin `knockout' dog
Authors/Creators: S.J. Schatzberg (Author/Creator) - North Carolina State University
N.J. Olby (Author/Creator) - North Carolina State University
M. Breen (Author/Creator) - Animal Health Trust
L.V.B. Anderson (Author/Creator) - Newcastle University
C.F. Langford (Author/Creator) - Wellcome Sanger Institute
H.F. Dickens (Author/Creator) - Animal Health Trust
S.D. Wilton (Author/Creator) - Australian Neuromuscular Research Institute, Medical Center, Nedlands, Western Australia 6009, Australia
C.J. Zeiss (Author/Creator) - Cornell University
M.M. Binns (Author/Creator) - Animal Health Trust
J.N. Kornegay (Author/Creator) - University of Missouri
G.E. Morris (Author/Creator) - MRIC Biochemistry Group, N.E. Wales Institute, Wrexham LL11 2AW, UK
N.J.H. Sharp (Author/Creator) - North Carolina State University
Publication Details: Neuromuscular Disorders, Vol.9(5), pp.289-295
Publisher: Elsevier BV
Identifiers: 991005541553707891
Copyright: 1999 Elsevier B.V.
Murdoch Affiliation: Murdoch University
Language: English
Resource Type: Journal article

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Collaboration types: Domestic collaboration; International collaboration
Citation topics: 1 Clinical & Life Sciences; 1.255 Musculoskeletal Disorders; 1.255.628 Duchenne Muscular Dystrophy
Web Of Science research areas: Clinical Neurology; Neurosciences
ESI research areas: Neuroscience & Behavior