Nuclear-magnetic-resonance spectroscopy studies of the flexible linkages between lipoyl domains in the pyruvate dehydrogenase multienzyme complex from Escherichia coli

1986 ◽  
Vol 14 (6) ◽  
pp. 1231-1232 ◽  
Author(s):  
SHEENA E. RADFORD ◽  
ERNEST D. LAUE ◽  
RICHARD N. PERHAM
Author(s):  
Douglas W. Zochodne ◽  
Wilma J. Koopman ◽  
Norbert J. Witt ◽  
Terry Thompson ◽  
Albert A. Driedger ◽  
...  

ABSTRACT:Five siblings with autosomal dominant oculopharyngeal muscular dystrophy (OPMD) underwent P-31 Nuclear Magnetic Resonance Spectroscopy studies of forearm flexor muscles. Mean values of PCr/(PCr + Pi) in the patients were reduced (p = 0.01) and pH elevated (p = 0.02) in resting muscle when compared to controls. During exercise PCr/(PCr + Pi) fell quickly to values less than controls (p < 0.0001) despite submaximal exercise output and developed exercise-induced acidosis which exceeded that of controls (p = 0.05). Acidosis recovered slowly despite relatively normal recovery of PCr/(PCr + Pi) following exercise. Within the patient group, however, one member had normal resting, exercise and recovery values. The studies suggest that OPMD is a more widespread disorder of striated muscle than clinically appreciated. The pattern of findings observed in OPMD differs from those identified in denervation, disuse and mitochondrial myopathy.


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