PMID- 1904072
OWN - NLM
STAT- MEDLINE
DCOM- 19910710
LR  - 20181113
IS  - 0021-9738 (Print)
IS  - 0021-9738 (Linking)
VI  - 87
IP  - 6
DP  - 1991 Jun
TI  - Structure of a monoclonal kappa chain of the V kappa IV subgroup in the kidney 
      and plasma cells in light chain deposition disease.
PG  - 2186-90
AB  - That structural abnormalities may be responsible for nonamyloid immunoglobulin 
      (Ig) light chain deposition disease (LCDD) is suggested by previous results of Ig 
      biosynthesis studies, but this hypothesis was not documented at the molecular 
      level. We report on the first complete primary sequence deduced from cDNA 
      analysis of a kappa light chain responsible for LCDD associated with an 
      apparently nonsecretory myeloma. Bone marrow myeloma cells contained 
      intracellular kappa chains and no heavy chains by immunofluorescence. Kidney 
      biopsy showed typical nonamyloid PAS-positive kappa chain deposits. SDS-PAGE 
      analysis of material extracted from a kidney biopsy specimen and of Ig produced 
      by the myeloma cells revealed kappa chains of abnormally high apparent molecular 
      mass (30,000). Comparison of the NH2-terminal aminoacid sequence of the kappa 
      chain deposited in the kidney and of the complete sequence of several identical 
      kappa cDNA clones from bone marrow cells showed the identity of the tissue 
      deposited and plasma cell kappa chain. The kappa mRNA had an overall normal 
      structure and corresponded to the V kappa IV gene rearranged to J kappa 1 and 
      followed by a normal constant exon of the Km(3) allotype. The variable sequence 
      differed from the V kappa IV germline gene by nine point mutations, including an 
      Asp----Asn substitution at position +70 resulting in a potential N-glycosylation 
      site. In vitro biosynthesis experiments and treatment with N-glycosidase provided 
      evidence for the intracellular glycosylation of the monoclonal kappa chain. The 
      peculiar sequence and the glycosylation of a kappa chain of the rare V kappa IV 
      subgroup might be responsible for structural abnormalities leading to tissue 
      deposition.
FAU - Cogne, M
AU  - Cogne M
AD  - Centre National de la Recherche Scientifique, URA 1172, University Hospital, 
      Poitiers, France.
FAU - Preud'homme, J L
AU  - Preud'homme JL
FAU - Bauwens, M
AU  - Bauwens M
FAU - Touchard, G
AU  - Touchard G
FAU - Aucouturier, P
AU  - Aucouturier P
LA  - eng
SI  - GENBANK/M38267
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Invest
JT  - The Journal of clinical investigation
JID - 7802877
RN  - 0 (Immunoglobulin Variable Region)
RN  - 0 (Immunoglobulin kappa-Chains)
SB  - IM
MH  - Amino Acid Sequence
MH  - Base Sequence
MH  - Blotting, Western
MH  - Gene Rearrangement, B-Lymphocyte, Light Chain
MH  - *Genes, Immunoglobulin
MH  - Humans
MH  - Hypergammaglobulinemia/*genetics
MH  - Immunoglobulin Variable Region/*genetics
MH  - Immunoglobulin kappa-Chains/*genetics
MH  - Kidney/*immunology
MH  - Molecular Sequence Data
PMC - PMC296978
EDAT- 1991/06/01 00:00
MHDA- 1991/06/01 00:01
PMCR- 1991/06/01
CRDT- 1991/06/01 00:00
PHST- 1991/06/01 00:00 [pubmed]
PHST- 1991/06/01 00:01 [medline]
PHST- 1991/06/01 00:00 [entrez]
PHST- 1991/06/01 00:00 [pmc-release]
AID - 10.1172/JCI115252 [doi]
PST - ppublish
SO  - J Clin Invest. 1991 Jun;87(6):2186-90. doi: 10.1172/JCI115252.