Document Detail


Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis.
MedLine Citation:
PMID:  20064157     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Serum amyloid P component (SAP) is a universal constituent of amyloid deposits and contributes to their formation and/or persistence. We therefore developed CPHPC ((R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexa-noyl]pyrrolidine-2 carboxylic acid), a novel bis(D-proline) drug, to specifically target SAP and report here a first, exploratory, open label proof of principle study in systemic amyloidosis. CPHPC produced sustained, >95% depletion of circulating SAP in all patients and c. 90% reduction in the SAP content of the two amyloidotic organs that became available. There were no significant adverse effects of either SAP depletion or CPHPC itself. No accumulation of amyloid was demonstrable by SAP scintigraphy in any patient on the drug. In hereditary fibrinogen amyloidosis, which is inexorably progressive, proteinuria was reduced in four of five patients receiving CPHPC and renal survival was prolonged compared to a historical control group. These promising clinical observations merit further study.
Authors:
Julian D Gillmore; Glenys A Tennent; Winston L Hutchinson; Janet Ruth Gallimore; Helen J Lachmann; Hugh J B Goodman; Mark Offer; David J Millar; Aviva Petrie; Philip N Hawkins; Mark B Pepys
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-01-08
Journal Detail:
Title:  British journal of haematology     Volume:  148     ISSN:  1365-2141     ISO Abbreviation:  Br. J. Haematol.     Publication Date:  2010 Mar 
Date Detail:
Created Date:  2010-05-04     Completed Date:  2010-06-29     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  0372544     Medline TA:  Br J Haematol     Country:  England    
Other Details:
Languages:  eng     Pagination:  760-7     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Amyloidosis / blood,  drug therapy*,  metabolism*
Carboxylic Acids / chemistry,  metabolism,  pharmacology,  therapeutic use*
Female
Humans
Male
Middle Aged
Proteinuria / drug therapy
Serum Amyloid P-Component / drug effects*,  metabolism*
Grant Support
ID/Acronym/Agency:
G7900510//Medical Research Council; G97900510//Medical Research Council
Chemical
Reg. No./Substance:
0/Carboxylic Acids; 0/Serum Amyloid P-Component

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


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