Expression of surrogate light chain in common variable immunodeficiency.
VpreB and lambda-like genes encode proteins that can associate to form a light-chain-like structure, the surrogate light chain (SLC). VpreB and lambda-like genes are selectively expressed in human progenitor B and precursor B (pre-B) cells. We analyzed the expression of VpreB and lambda-like genes in seven patients with common variable immunodeficiency (CVI). Gene expression was examined in the peripheral blood mononuclear cells (PBMCs) using the polymerase chain reaction. In four CVI patients, two of whom were brothers, the expression of the VpreB gene was detectable in the PBMCs. In the brothers with CVI, the expression of the VpreB gene was also examined in bone marrow cells, where it was found to be expressed in trace amounts. A number of the brothers' bone marrow cells were stained with mu and kappa antibodies using the avidin-biotin complex method. In lambda 5-gene-deleted mice, B cell development in the bone marrow is blocked at the pre-B stage, but the blockade is leaky. We suggest that expression of the SLC is weak in patients with CVI compared with their normal counterparts, and this may be one of the underlying mechanisms of CVI.
Yano
M
M
Department of Pediatrics, Gifu University School of Medicine, Japan.
Kondo
N
N
Kato
Y
Y
Shinoda
S
S
Inoue
R
R
Orii
T
T
D000328:Adult; D000361:Agammaglobulinemia / Q000235:genetics*; D001483:Base Sequence; D001853:Bone Marrow / Q000276:immunology; D002648:Child; D017931:DNA Primers / Q000737:chemistry; D005260:Female; D015870:Gene Expression; D005803:Genes, Immunoglobulin; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000235:genetics*; D054444:Immunoglobulin Light Chains, Surrogate; D007148:Immunoglobulin mu-Chains / Q000235:genetics*; D016130:Immunophenotyping; D007963:Leukocytes, Mononuclear / Q000276:immunology; D008297:Male; D008562:Membrane Glycoproteins / Q000235:genetics*; D008969:Molecular Sequence Data; D015183:Restriction Mapping
D017931:DNA Primers; D007147:Immunoglobulin Light Chains; D054444:Immunoglobulin Light Chains, Surrogate; D007148:Immunoglobulin mu-Chains; D008562:Membrane Glycoproteins