Document Detail


IgM pyroglobulinemia with erythrocytosis presenting as hyperviscosity syndrome. II. Biochemical properties and mechanisms of pyrogel formation.
MedLine Citation:
PMID:  822709     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
An immunoglobulin M with kappa light chains (IgMK) pyroglobulin from a patient with hyperviscosity syndrome, erythrocytosis and coagulation defects has been studied for its immunochemical properties. At physiologic temperatures the purified macropyroglobulin showed a striking tendency to aggregate in the pentamer as well as in the monomer form. This property was also observed in its H chains. Aggregate formation of the pentamers may have contributed to the blood viscosity and coagulation defects. Formation of pyrogel at 56degreesC was observed with pentamers as well as monomers, but not with separated H or L chains. Amino acid analysis showed quantitative abnormalities of aspartic acid, glycine, cystine and leucine within the H chains. Solubility of the pyrogel in sodium dodecyl sulfate, the pyroglobulin's tendency to aggregate and the cystine deficit of H chains implicate conformational changes leading to hydrophobic bonding at 56degreesC in the formation of pyrogel.
Authors:
S R McCann; H H Zinneman; M M Oken; A A Sinha
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of medicine     Volume:  61     ISSN:  0002-9343     ISO Abbreviation:  Am. J. Med.     Publication Date:  1976 Sep 
Date Detail:
Created Date:  1976-10-29     Completed Date:  1976-10-29     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  0267200     Medline TA:  Am J Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  321-5     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Amino Acids / analysis
Blood Viscosity*
Gels
Humans
Immunoglobulin M* / metabolism
Immunoglobulin kappa-Chains
Paraproteinemias / complications,  diagnosis,  metabolism*
Paraproteins* / metabolism
Polycythemia / complications*
Pyroglobulins* / metabolism
Waldenstrom Macroglobulinemia / metabolism*
Chemical
Reg. No./Substance:
0/Amino Acids; 0/Gels; 0/Immunoglobulin M; 0/Immunoglobulin kappa-Chains; 0/Paraproteins; 0/Pyroglobulins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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