Biased immunoglobulin G (IgG) subclass production in a case of hyper-IgM syndrome.
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.
McLean
G R
GR
The Biomedical Research Centre, University of British Columbia, 2222 Health Sciences Mall, Vancouver, BC, Canada V6T 1Z3. gmclean@brc.ubc.ca
Miller
K K
KK
Schrader
J W
JW
Junker
A K
AK
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
D007127:Immunoglobulin Constant Regions; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007135:Immunoglobulin Variable Region; D007148:Immunoglobulin mu-Chains