Muscle dystrophy

Structure Rat foetal resorption Muscle dystrophy in chickens Haemolysis in rats... 

Long stimulus trains will also induce tetanic fade, observed as a decrease in force during a single contraction. Fade is measured as the ratio of final force to initial peak force. Diseased muscle/nerve may show a lower ratio. Accompanying measurement of EMG can reveal if fade is due to failure of the muscle or the nerve. In purely muscle defects (muscle dystrophy) force will drop without any change in EMG whereas reduced force accompanied by reduced EMG suggests a defect in transmission (NMJ) (e.g., (3)) or excitation-contraction coupling. 

Muscle dystrophies, neurodegenerative, neuromuscular junction diseases M, N... 

In healthy people the rate of ketogenesis, and therefore the concentration of acetoacetate and 3-hydroxybutyrate in the blood, will decrease after meals, but may increase in mitochondrial disorders [15, 20]. Increased serum ammonia, creatine kinase or CSF protein concentration is not indicative for a mitochondrial disturbance. If found, urea cycle defects, liver cirrhosis, muscle dystrophy or brain necrosis must be considered. Patients with Kearns-Sayre syndrome and Leigh syndrome, however, often have increased protein concentrations in the CSF. 

Under normal conditions, AChE-S is an essential component of functional NMJ, but AChE-R is not. Compatible with this notion, the loss of the ColQ collagen-like structural subunit linking AChE-S to the NMJ leads to congenital myasthenia (Engel, 2012), and modified AChE levels were associated with muscle dystrophy in both chickens and mice (Comim et al., 2011 Vidal et al., 2013). 

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