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Compromised Exercise Capacity and Mitochondrial Dysfunction in the Osteogenesis Imperfecta Murine (oim) Mouse Model
Journal of bone and mineral research, 2019-09, Vol.34 (9), p.1646-1659
Gremminger, Victoria L
Jeong, Youngjae
Cunningham, Rory P
Meers, Grace M
Rector, R Scott
Phillips, Charlotte L
2019
Details
Autor(en) / Beteiligte
Gremminger, Victoria L
Jeong, Youngjae
Cunningham, Rory P
Meers, Grace M
Rector, R Scott
Phillips, Charlotte L
Titel
Compromised Exercise Capacity and Mitochondrial Dysfunction in the Osteogenesis Imperfecta Murine (oim) Mouse Model
Ist Teil von
Journal of bone and mineral research, 2019-09, Vol.34 (9), p.1646-1659
Ort / Verlag
United States: Wiley Subscription Services, Inc
Erscheinungsjahr
2019
Link zum Volltext
Quelle
Wiley Online Library All Journals
Beschreibungen/Notizen
ABSTRACT Osteogenesis imperfecta (OI) is a heritable connective tissue disorder that most often arises from type I collagen—COL1A1 and COL1A2—gene defects leading to skeletal fragility, short stature, blue‐gray sclera, and muscle weakness. Relative to the skeletal fragility, muscle weakness is much less understood. Recent investigations into OI muscle weakness in both patients and mouse models have revealed the presence of an inherent muscle pathology. Understanding the mechanisms responsible for OI muscle weakness is critical, particularly in light of the extensive cross‐talk between muscle and bone via mechanotransduction and biochemical signaling. In the following study we initially subjected WT and oim/oim mice, modeling severe human OI type III, to either weight‐bearing (voluntary wheel‐running) or non‐weight‐bearing (swimming) exercise regimens as a modality to improve muscle strength and ultimately bone strength. The oim/oim mice ran only 35% to 42% of the distance run by age‐ and sex‐matched WT mice and exhibited little improvement with either exercise regimen. Upon further investigation, we determined that oim/oim gastrocnemius muscle exhibited severe mitochondrial dysfunction as characterized by a 52% to 65% decrease in mitochondrial respiration rates, alterations in markers of mitochondrial biogenesis, mitophagy, and the electron transport chain components, as well as decreased mitochondrial citrate synthase activity, relative to age‐ and sex‐matched WT gastrocnemius muscle. Thus, mitochondrial dysfunction in the oim/oim mouse likely contributes to compromised muscle function and reduced physical activity levels. © 2019 American Society for Bone and Mineral Research.
Sprache
Englisch
Identifikatoren
ISSN: 0884-0431
eISSN: 1523-4681
DOI: 10.1002/jbmr.3732
Titel-ID: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_6744299
Format
–
Schlagworte
Animal models
,
Animals
,
Biomarkers - metabolism
,
Bone and Bones - pathology
,
Bone and Bones - physiopathology
,
BONE QCT/MICROCT
,
Bone strength
,
Citrate synthase
,
Collagen (type I)
,
Connective tissue diseases
,
Disease Models, Animal
,
DNA, Mitochondrial - metabolism
,
Electron Transport
,
Electron transport chain
,
EXERCISE
,
Female
,
Gastrocnemius muscle
,
GENETIC ANIMAL MODELS
,
Glycogen - metabolism
,
Male
,
Mechanotransduction
,
Membrane Proteins - metabolism
,
Mice, Inbred C57BL
,
Microtubule-Associated Proteins - metabolism
,
Mitochondria
,
Mitochondria - pathology
,
Mitochondrial Proteins - metabolism
,
Mitophagy
,
Muscle strength
,
Muscles - ultrastructure
,
Organ Size
,
Organelle Biogenesis
,
Osteogenesis
,
OSTEOGENESIS IMPERFECTA
,
Osteogenesis Imperfecta - physiopathology
,
Physical activity
,
Physical Conditioning, Animal
,
Physical fitness
,
Physical training
,
SKELETAL MUSCLE
,
Swimming
,
Vitamin D
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