title: Increased pro-opiomelanocortin-derived peptide release in myotonic dystrophy.
abstract: 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.
authors.affiliation: Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.
authors.forename: R V
authors.initials: RV
authors.lastname: Jackson
authors.forename: G A
authors.initials: GA
authors.lastname: Fleming
authors.forename: C R
authors.initials: CR
authors.lastname: Sussman
authors.forename: A B
authors.initials: AB
authors.lastname: Atkinson
authors.forename: G S
authors.initials: GS
authors.lastname: Decherney
authors.forename: C R
authors.initials: CR
authors.lastname: Debold
authors.forename: D R
authors.initials: DR
authors.lastname: Davis
authors.forename: W E
authors.initials: WE
authors.lastname: Nicholson
authors.forename: D P
authors.initials: DP
authors.lastname: Island
authors.forename: D
authors.initials: D
authors.lastname: Rabin
mesh_terms: 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
chemical_list: D007328:Insulin; D010455:Peptides; D001615:beta-Endorphin; D011333:Pro-Opiomelanocortin; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone; D006854:Hydrocortisone