Increased pro-opiomelanocortin-derived peptide release in myotonic dystrophy.
The response of plasma immunoreactive (IR)-ACTH, IR-beta-endorphin (beta-END) and IR-cortisol to insulin-induced hypoglycaemia, an acute stimulus to the pituitary corticotrophs through the central nervous system, and to synthetic ovine corticotrophin-releasing hormone (CRH), a direct corticotroph stimulator, were studied in normal males and males with myotonic dystrophy. Myotonics had an increased IR-ACTH and IR-beta-END response to hypoglycaemia and an increased IR-ACTH response to CRH compared with normals. Plasma IR-cortisol response were not different in either group of subjects to both stimuli. This neuroendocrine abnormality in myotonic dystrophy may represent a manifestation of the purported specific cell membrane defect underlying the disease. This is the first report of an abnormality in proopiomelanocortin peptide release in myotonic dystrophy.
Jackson
R V
RV
Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
Fleming
G A
GA
Sussman
C R
CR
Atkinson
A B
AB
Decherney
G S
GS
Debold
C R
CR
Davis
D R
DR
Nicholson
W E
WE
Island
D P
DP
Rabin
D
D
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D003346:Corticotropin-Releasing Hormone; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007328:Insulin; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000097:blood* / Q000175:diagnosis; D010455:Peptides / Q000097:blood*; D011333:Pro-Opiomelanocortin / Q000097:blood*; D001615:beta-Endorphin / Q000097:blood
D007328:Insulin; D010455:Peptides; D001615:beta-Endorphin; D011333:Pro-Opiomelanocortin; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D006854:Hydrocortisone