id:"swepub:oai:DiVA.org:umu-91978"
Search: id:"swepub:oai:DiVA.org:umu-91978" > Wnt and Extraocular...
- 1 of 1
- Previous record
- Next record
- To hitlist
Wnt and Extraocular Muscle Sparing in Amyotrophic Lateral Sclerosis
-
- McLoon, Linda K (author)
- Umeå universitet,Anatomi,Department of Ophthalmology and Visual Neurosciences, University of Minnesota, 6 Minneapolis, MN 55455
-
- Harandi, Vahid M (author)
- Umeå universitet,Anatomi
-
- Brännstrom, Thomas (author)
- Umeå universitet,Patologi
- show more...
- show less...
- (creator_code:org_t)
- ARVO, The Association for Reserach in Vision and Ophthalmology, 2014
- 2014
- English.
- In: Investigative Ophthalmology and Visual Science. - : ARVO, The Association for Reserach in Vision and Ophthalmology. - 0146-0404 .- 1552-5783. ; 55:9, s. 5482-5496
- Journal article (peer-reviewed)
Abstract
Subject headings
Close
- PURPOSE: The extraocular muscles (EOM) and their motor neurons are spared in amyotrophic lateral sclerosis (ALS). In limb muscle axon retraction from the neuromuscular junctions occurs early in the disease. Wnts, a conserved family of secreted signaling molecules, play a critical role in neuromuscular junction formation. This is the first study to examine Wnt signaling for its potential involvement in maintenance of normal morphology in EOMs in ALS.METHODS: EOM and limb muscle axons, neuromuscular junctions, and myofibers from control, aging, and ALS patients and the SOD1G93A mouse model of ALS were quantified for their expression of Wnt1, Wnt3a, Wnt5a, Wnt7a, and beta-catenin.RESULTS: All four Wnt isoforms were expressed in most axon profiles in all human EOMs. Significantly fewer were positive for Wnt1, Wnt3a, and Wnt7a in the human limb muscles. Similar differential patterns in Wnt myofiber expression was also seen, except for Wnt7a, where expression was elevated. In the SOD1G93A mouse, all 4 Wnt isoforms were significantly decreased in the neuromuscular junctions at the terminal stage compared to age matched controls. Beta-catenin was activated in a subset of myofibers in EOM and limb muscle in all patients.CONCLUSIONS: The differences in Wnt expression in EOM and limb muscle, particularly at the neuromuscular junction level, suggest that they play a role in the pathophysiology of ALS. Collectively, the data support a role for Wnt signaling in the preservation of the EOM in ALS and their dysregulation and the subsequent development of pathology in the ALS limb muscles.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Oftalmologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Ophthalmology (hsv//eng)
Keyword
- extraocular muscles
- Wnt
- neuromuscular junctions
- amyotrophic lateral sclerosis
- beta-catenin
- skeletal muscle
- SOD1(G93A) mice
Publication and Content Type
- ref (subject category)
- art (subject category)
Find in a library
- Investigative Ophthalmology and Visual Science (Search for host publication in LIBRIS)
To the university's database
- 1 of 1
- Previous record
- Next record
- To hitlist
Find more in SwePub
- By the author/editor
- McLoon, Linda K
- Harandi, Vahid M
- Brännstrom, Thom ...
- Andersen, Peter ...
- Liu, Jing-Xia
- About the subject
-
- MEDICAL AND HEALTH SCIENCES
- MEDICAL AND HEAL ...
- and Clinical Medicin ...
- and Ophthalmology
- Articles in the publication
- Investigative Op ...
- By the university
- Umeå University
Search outside SwePub
- Extend your search to:
- Google Book Search
- Google Scholar
Kungliga biblioteket hanterar dina personuppgifter i enlighet med EU:s dataskyddsförordning (2018), GDPR. Läs mer om hur det funkar här.
Så här hanterar KB dina uppgifter vid användning av denna tjänst.
- Copyright © LIBRIS - National Library Systems
- LIBRIS.kb.se
