title: Increased plasma membrane fluidity and decreased receptor availability of nonmuscle cells in myotonic dystrophy.
abstract: Plasma membrane fluidity of intact nonmuscle cells from patients with myotonic dystrophy (MyD) was determined by fluorescence anisotropy measurements. Anisotropy values of the probe diphenylhexatriene were decreased in patient mononuclear cells (0.163 +/- 0.017, n = 13) versus controls (0.181 +/- 0.013, n = 13, P less than 0.01) and in patient platelets (0.087 +/- 0.017, n = 9) versus controls (0.137 +/- 0.015, n = 9, P less than 0.001) indicating increased plasma membrane fluidity in patient nonmuscle cells. Vasopressin plasma concentrations were increased in patients (7.4 +/- 2.1 pg/ml, n = 12) versus controls (4.5 +/- 1.4 pg/ml, n = 22, P less than 0.0005), whereas serum osmolality was normal. These data are compatible with a decreased vasopressin sensitivity in MyD patients. Specific binding of 125I-labelled vasoactive intestinal peptide (VIP) was decreased in patient mononuclear cells (2.9 +/- 0.9%/10(6) cells, n = 8) versus controls (5.2 +/- 1.6%/10(6) cells, n = 9, P less than 0.005) and receptor affinity for VIP was decreased in patient mononuclear cells (Kd = 0.26 +/- 0.05 nM, n = 8) versus controls (Kd = 0.19 +/- 0.02 nM, n = 9, P less than 0.005). In nonmuscle cells of MyD patients, increased membrane fluidity correlated with decreased receptor availability. This might explain the various endocrine defects described in MyD patients.
authors: Department of Pediatrics, University of Hamburg, F.R.G.
authors: C
authors: C
authors: Hübner
authors: S G
authors: SG
authors: Lindner
authors: M
authors: M
authors: Albani
authors: M
authors: M
authors: Ballmann
authors: B
authors: B
authors: Keup
authors: M
authors: M
authors: Schürmann
authors: H
authors: H
authors: Stegner
authors: M
authors: M
authors: Claussen
authors: A
authors: A
authors: Kohlschütter
mesh_terms: D000293:Adolescent; D000328:Adult; D001127:Arginine Vasopressin / Q000097:blood; D001792:Blood Platelets / Q000502:physiology*; D005260:Female; D006801:Humans; D007963:Leukocytes, Mononuclear / Q000502:physiology*; D008297:Male; D008560:Membrane Fluidity; D008563:Membrane Lipids / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000097:blood* / Q000378:metabolism; D014660:Vasoactive Intestinal Peptide / Q000097:blood
chemical_list: D008563:Membrane Lipids; D001127:Arginine Vasopressin; D014660:Vasoactive Intestinal Peptide