Accelerated breakdown of immunoglobulin G (IgG) in myotonic dystrophy: a hereditary error of immunoglobulin catabolism.
Year: 1966
Type: article
Abstract: Myotonic dystrophy is a hereditary progressive muscular abnormality with dominant transmittance that was first proposed as a separate entity by Batten and Gibb (1) and by Steinert (2) in 1909.The muscular abnormality that is the dominant feature of the disease is characterized by weakness, wasting, and myotonia, especially of the facial, neck, and distal musculature.Other abnormalities frequently associated with the disease include frontal alopecia, cataracts, gonadal atrophy, low basal metabolic rate with normal thyroid function, impaired glucose tolerance, and electrocardiographic abnormalities.Reduction in serum y-globulin concentration has been observed in patients with myotonic dystrophy by some workers (3, 4) but not by others (5).In 1956 Zinneman and Rotstein reported decreased y-globulins in 7 of 12 patients with myotonic dystrophy (6).It was found that the survival half- time of 1311-labeled y-globulin in six of these patients averaged 7.6 days compared to 10.5 days in controls, whereas the survival of albumin-'31I averaged 10.2 days compared to 9.6 days in con- trols.In recent years, the plasma proteins that possess immunologic activity have been the subject of in- tensive study.These proteins as a group are re- ferred to as immunoglobulins and can be divided into at least three major classes.These are IgG (7 S y2-globulins), IgA (,82A-globulins), and IgM (yi-macroglobulins or f82M-globulins).Each of these fractions possesses antibody activity and cer-
DOI: 10.1172/jci105346
Authors: R D Wochner, G Drews, W Strober, T A Waldmann
Language: English
Subfield: Cellular and Molecular Neuroscience
Field: Neuroscience
Domain: Life Sciences
Open Access status: bronze
OpenAlex ID: W2021196599