Alpha-Adrenergic Receptors in Familial Amyloidotic Polyneuropathy
Type 1 familial amyloidotic polyneuropothy (FAP) is a rare hereditary amyloidosis with special involvement of the peripheral nervous system. Autonomic nerve involvement is a striking feature of this type. Constipation, explosive diarrhea, sexual impotence, sphincter impairment, hypohidrosis, orthostatic hypotension, and disordered cardiac conduction are commonly observed. Autonomic involvement is often responsible for death. In earlier studies, we found that patients with advanced FAP had decreased urinary excretion rates of norepinephrine (NE) (Suzuki et al., 1979) and decreased plasma NE concentration (Suzuki et al., 1981), and that the responsiveness of their blood pressure to infused NE was exaggerated (Suzuki et al,. 1980a). These findings suggested that in advanced FAP patients noradrenergic receptors are more sensitive to agonists because NE in the sympathetic nerves is chronically depleted. We now report the findings of α2-adrenergic receptor binding in platelet membranes, and of α1-adrenergic receptor binding in aortic membranes from the FAP patients.
KeywordsOrthostatic Hypotension Adrenergic Receptor Platelet Membrane Liquid Scintillation Spectrometer Familial Amyloid Polyneuropathy
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