Vacuolar degeneration of skeletal muscle in transgenic mice overexpressing ORP150.
نویسندگان
چکیده
ORP150 is a hypoxic stress-induced protein located in the endoplasmic reticulum. Transgenic mice overexpressing ORP150 (ORP-Tg) exhibit vacuolar degeneration in the heart. To determine whether vacuolization is present in skeletal muscle, we pathologically examined ORP-Tg mice. After 60 days of age, severe vacuolization was found in the soleus muscles of the hind legs of the ORP-Tg mice. Immunohistochemical staining of ORP150 revealed co-localization of ORP150 and vacuolization in the affected cells. Electron microscopy revealed a marked increase in the number of rough-surfaced endoplasmic reticula (rER) and distention of the cisterna. These findings suggest that overexpression of ORP150 causes accumulation of ORP150 in the rER, resulting in vacuolar degeneration in the skeletal muscle of ORP-Tg mice.
منابع مشابه
Injury to skeletal muscle of mice following acute and sub-acute pregabalin exposure
Objective(s): Pregabalin (PGB) is a new antiepileptic drug that has received FDA approval for patient who suffers from central neuropathic pain, partial seizures, generalized anxiety disorder, fibromyalgia and sleep disorders. This study was undertaken to evaluate the possible adverse effects of PGB on the muscular system of mice. Materials and Methods: To evaluate the effect of PGB on skeletal...
متن کاملCentronuclear myopathy in mice lacking a novel muscle-specific protein kinase transcriptionally regulated by MEF2.
Myocyte enhancer factor 2 (MEF2) plays essential roles in transcriptional control of muscle development. However, signaling pathways acting downstream of MEF2 are largely unknown. Here, we performed a microarray analysis using Mef2c-null mouse embryos and identified a novel MEF2-regulated gene encoding a muscle-specific protein kinase, Srpk3, belonging to the serine arginine protein kinase (SRP...
متن کاملMechanisms mediating insulin resistance in transgenic mice overexpressing mouse apolipoprotein A-II.
We previously demonstrated that transgenic mice overexpressing mouse apolipoprotein A-II (apoA-II) exhibit several traits associated with the insulin resistance (IR) syndrome, including increased atherosclerosis, hypertriglyceridemia, obesity, and IR. The skeletal muscle appeared to be the insulin-resistant tissue in the apoA-II transgenic mice. We now demonstrate a decrease in FA oxidation in ...
متن کاملFHL1 Reduces Dystrophy in Transgenic Mice Overexpressing FSHD Muscular Dystrophy Region Gene 1 (FRG1)
Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal-dominant disease with no effective treatment. The genetic cause of FSHD is complex and the primary pathogenic insult underlying the muscle disease is unknown. Several disease candidate genes have been proposed including DUX4 and FRG1. Expression analysis studies of FSHD report the deregulation of genes which mediate myoblast differen...
متن کاملSkeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of motor neurons (MNs) that causes skeletal muscle paralysis. Familial forms of ALS are linked to mutations in the superoxide dismutase-1 (SOD1) gene. The mechanisms of human SOD1 (hSOD1) toxicity to MNs are unknown. We hypothesized that skeletal muscle is a primary site of pathogenesis in ALS that triggers MN degeneration...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- The Journal of veterinary medical science
دوره 70 1 شماره
صفحات -
تاریخ انتشار 2008