title: Familial properdin deficiency associated with chronic discoid lupus erythematosus.
abstract: A large family comprised of 18 members is described. Four male members are properdin-deficient, all are healthy bar the index patient who presented with chronic discoid lupus erythematosus. Serum from properdin-deficient males had a reduced ability to lyse rabbit erythrocytes via the alternative pathway or solubilize pre-formed immune complexes. Addition of purified properdin restored these activities. Classical pathway activity was normal. Definite, probable and possible female carriers had normal classical and alternative pathway activities.
authors.affiliation: University of Glasgow, Department of Pathology.
authors.forename: E R
authors.initials: ER
authors.lastname: Holme
authors.forename: J
authors.initials: J
authors.lastname: Veitch
authors.forename: A
authors.initials: A
authors.lastname: Johnston
authors.forename: G
authors.initials: G
authors.lastname: Hauptmann
authors.forename: B
authors.initials: B
authors.lastname: Uring-Lambert
authors.forename: M
authors.initials: M
authors.lastname: Seywright
authors.forename: V
authors.initials: V
authors.lastname: Docherty
authors.forename: W N
authors.initials: WN
authors.lastname: Morley
authors.forename: K
authors.initials: K
authors.lastname: Whaley
mesh_terms: D005190:Family; D006801:Humans; D008179:Lupus Erythematosus, Discoid / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011414:Properdin / Q000172:deficiency*
chemical_list: D011414:Properdin