title: Biased immunoglobulin G (IgG) subclass production in a case of hyper-IgM syndrome.
abstract: Hyper-immunoglobulin M (IgM) syndrome (HIGM) is a rare heterogeneous primary immune deficiency. We describe a patient with HIGM characterized by skewed production of serum IgG subclasses and normal somatic hypermutation. This case may represent a subgroup of HIGM type 4 that is characterized by a biased switching to the V-region proximal constant regions.
authors.affiliation: The Biomedical Research Centre, University of British Columbia, 2222 Health Sciences Mall, Vancouver, BC, Canada V6T 1Z3. gmclean@brc.ubc.ca
authors.forename: G R
authors.initials: GR
authors.lastname: McLean
authors.forename: K K
authors.initials: KK
authors.lastname: Miller
authors.forename: J W
authors.initials: JW
authors.lastname: Schrader
authors.forename: A K
authors.initials: AK
authors.lastname: Junker
mesh_terms: D000293:Adolescent; D005260:Female; D015322:Gene Rearrangement, B-Lymphocyte / Q000235:genetics*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood* / Q000235:genetics* / Q000473:pathology; D007127:Immunoglobulin Constant Regions / Q000235:genetics; D007074:Immunoglobulin G / Q000097:blood*; D007075:Immunoglobulin M / Q000097:blood / Q000235:genetics*; D007135:Immunoglobulin Variable Region / Q000235:genetics; D007148:Immunoglobulin mu-Chains / Q000235:genetics
chemical_list: D007127:Immunoglobulin Constant Regions; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007135:Immunoglobulin Variable Region; D007148:Immunoglobulin mu-Chains