Document Detail

Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis.
MedLine Citation:
PMID:  20064157     Owner:  NLM     Status:  MEDLINE    
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.
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
Related Documents :
12911167 - Glomerulopathy in kuwait: the spectrum over the past 7 years.
86667 - The loin-pain/haematuria syndrome.
10878397 - The urinary podocyte as a marker for the differential diagnosis of idiopathic focal glo...
7034297 - Immunofluorescent studies on acute phase reactants in patients with various types of ch...
25052157 - Traumatic brain injury associated coagulopathy.
17988217 - Association of low-grade inflammation and platelet activation in patients with hyperten...
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    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Amyloidosis / blood,  drug therapy*,  metabolism*
Carboxylic Acids / chemistry,  metabolism,  pharmacology,  therapeutic use*
Middle Aged
Proteinuria / drug therapy
Serum Amyloid P-Component / drug effects*,  metabolism*
Grant Support
G7900510//Medical Research Council; G97900510//Medical Research Council
Reg. No./Substance:
0/Carboxylic Acids; 0/Serum Amyloid P-Component

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

Previous Document:  Blasts in transient leukaemia in neonates with Down syndrome differentiate into basophil/mast-cell a...
Next Document:  KIT mutations confer a distinct gene expression signature in core binding factor leukaemia.