The golden retriever model of Duchenne muscular dystrophy

Skeletal Muscle
Joe N Kornegay

Abstract

Duchenne muscular dystrophy (DMD) is an X-linked disease caused by mutations in the DMD gene and loss of the protein dystrophin. The absence of dystrophin leads to myofiber membrane fragility and necrosis, with eventual muscle atrophy and contractures. Affected boys typically die in their second or third decade due to either respiratory failure or cardiomyopathy. Despite extensive attempts to develop definitive therapies for DMD, the standard of care remains prednisone, which has only palliative benefits. Animal models, mainly the mdx mouse and golden retriever muscular dystrophy (GRMD) dog, have played a key role in studies of DMD pathogenesis and treatment development. Because the GRMD clinical syndrome is more severe than in mice, better aligning with the progressive course of DMD, canine studies may translate better to humans. The original founder dog for all GRMD colonies worldwide was identified in the early 1980s before the discovery of the DMD gene and dystrophin. Accordingly, analogies to DMD were initially drawn based on similar clinical features, ranging from the X-linked pattern of inheritance to overlapping histopathologic lesions. Confirmation of genetic homology between DMD and GRMD came with identification of th...Continue Reading

References

Sep 1, 1976·Archives of Neurology·J E Desmedt, S Borenstein
Jul 1, 1992·Journal of the Neurological Sciences·F P GaschenL K Pearce
Feb 1, 1992·American Journal of Physical Medicine & Rehabilitation·G T CarterR K Entrikin
Mar 1, 1991·Journal of the American College of Cardiology·N S MoiseR F Gilmour
Mar 1, 1990·International Journal of Cardiology·G NigroR J Bain
Jun 1, 1990·Journal of the Neurological Sciences·B A ValentineA de Lahunta
Mar 16, 1989·Nature·K P Campbell, S D Kahl
Oct 1, 1989·Journal of Child Neurology·J A Witkowski
Oct 1, 1989·Journal of Child Neurology·V Dubowitz
Oct 1, 1989·Journal of Child Neurology·J Lewis
Jul 1, 1985·Proceedings of the National Academy of Sciences of the United States of America·L M KunkelS A Latt
Oct 1, 1988·Muscle & Nerve·J N KornegayD C Levesque
Nov 1, 1988·The Journal of Heredity·B J CooperP W Concannon
Dec 1, 1988·Journal of the Neurological Sciences·B A ValentineJ T Blue
Jul 1, 1988·Journal of Veterinary Internal Medicine·B A ValentineD M Noden
Dec 1, 1986·Journal of Medical Genetics·Y BoydI Craig
Dec 1, 1985·Immunological Reviews·R Storb, E D Thomas
Jan 1, 1974·Zentralblatt für Veterinärmedizin. Reihe A·G H WentinkH J Hendriks
Dec 24, 1973·JAMA : the Journal of the American Medical Association·T L MunsatW Fowler
Feb 1, 1984·Proceedings of the National Academy of Sciences of the United States of America·G BulfieldK J Moore
Apr 1, 1984·Journal of the Neurological Sciences·D Gardner-Medwin, H M Johnston
Sep 1, 1995·American Journal of Physical Medicine & Rehabilitation·C M McDonaldB J Sigford
Sep 1, 1994·Neuromuscular Disorders : NMD·N J WinandB J Cooper
Sep 1, 1993·Neuromuscular Disorders : NMD·L V Nicholson
May 1, 1993·Archives of Disease in Childhood·L V NicholsonD Gardner-Medwin

❮ Previous
Next ❯

Citations

Aug 15, 2019·Journal of the American Heart Association·Lee-Jae GuoChristopher F Spurney
Aug 28, 2019·Journal of Neuromuscular Diseases·Inès BarthélémyLaurent Tiret
Feb 1, 2020·Expert Opinion on Drug Discovery·Nalinda B WasalaDongsheng Duan
Mar 28, 2020·Genes·Kenji Rowel Q LimToshifumi Yokota
Sep 25, 2017·FASEB Journal : Official Publication of the Federation of American Societies for Experimental Biology·Xingcai CaiGang Shu
Feb 19, 2020·Disease Models & Mechanisms·Jennifer Morgan, Terence Partridge
Feb 9, 2019·Human Genetics·Peter P Nghiem, Joe N Kornegay
May 31, 2019·Nature Reviews. Neurology·Ingrid E C Verhaart, Annemieke Aartsma-Rus
Apr 5, 2020·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Sara Mata LópezPeter P Nghiem
Jul 22, 2020·Pharmacological Reports : PR·Agnieszka Łoboda, Józef Dulak
Feb 17, 2019·Cellular and Molecular Life Sciences : CMLS·Paulina PodkalickaAgnieszka Loboda
Apr 1, 2020·Disease Models & Mechanisms·Miranda D GroundsPeter G Arthur
Oct 20, 2020·Expert Opinion on Biological Therapy·Anne-Fleur E Schneider, Annemieke Aartsma-Rus
Jan 28, 2021·Veterinary Medicine and Science·Jessica S FortinDongsheng Duan
Jan 5, 2021·JOR Spine·Naomi N LeeJames L Cook
Jan 8, 2021·Skeletal Muscle·James R MickelsonG Diane Shelton
Nov 30, 2020·Topics in Companion Animal Medicine·C SalvadoriC Cantile
Apr 7, 2021·British Journal of Pharmacology·Tibor HornyikKatja E Odening
Jun 3, 2021·Genes·Vidhya JagannathanGuo-Dong Wang
Jun 26, 2021·Pflügers Archiv : European journal of physiology·Barbora SvobodovaVladimir Rotrekl
Jul 14, 2021·Cardiovascular Research·Francesco CanonicoDomenico D'Amario
Aug 14, 2021·Neuromuscular Disorders : NMD·Natasha L HornbyRichard J Piercy
Jun 25, 2020·Molecular Therapy. Methods & Clinical Development·Isabel PunzónStéphane Blot
Nov 20, 2021·Disease Models & Mechanisms·Michael StirmEckhard Wolf

❮ Previous
Next ❯

Methods Mentioned

BETA
antisense oligonucleotide
light microscopy
biopsy
GTPase
antisense oligonucleotides

Clinical Trials Mentioned

NCT02090959

Software Mentioned

Ensembl
GRMD

Related Concepts

Related Feeds

Cardiomyopathy

Cardiomyopathy is a disease of the heart muscle, that can lead to muscular or electrical dysfunction of the heart. It is often an irreversible disease that is associated with a poor prognosis. There are different causes and classifications of cardiomyopathies. Here are the latest discoveries pertaining to this disease.