Role of Serum Amyloid P Component for Systemic Amyloidosis in Transgenic Mice Carrying Human Mutant Transthyretin Gene

Gerontology ◽  
1991 ◽  
Vol 37 (1) ◽  
pp. 56-62 ◽  
Author(s):  
F. Tashiro ◽  
S. Yi ◽  
S. Wakasugi ◽  
S. Maeda ◽  
K. Shimada ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Serum Amyloid ◽  
Systemic Amyloidosis ◽  
Amyloid P Component ◽  
Serum Amyloid P ◽  
Serum Amyloid P Component ◽  
Amyloid P

scholarly journals Role of serum amyloid P component in bacterial infection: Protection of the host or protection of the pathogen

2000 ◽  
Vol 97 (26) ◽  
pp. 14584-14589 ◽  
Author(s):  
M. Noursadeghi ◽  
M. C. M. Bickerstaff ◽  
J. R. Gallimore ◽  
J. Herbert ◽  
J. Cohen ◽  
...  
Keyword(s):  
Bacterial Infection ◽  
Serum Amyloid ◽  
Amyloid P Component ◽  
Serum Amyloid P ◽  
Serum Amyloid P Component ◽  
Amyloid P

scholarly journals Diagnostic Performance and Prognostic Value of Extravascular Retention of 123I-Labeled Serum Amyloid P Component in Systemic Amyloidosis

2007 ◽  
Vol 48 (6) ◽  
pp. 865-872 ◽  
Author(s):  
B. P.C. Hazenberg ◽  
M. H. van Rijswijk ◽  
M. N. Lub-de Hooge ◽  
E. Vellenga ◽  
E. B. Haagsma ◽  
...  
Keyword(s):  
Prognostic Value ◽  
Diagnostic Performance ◽  
Serum Amyloid ◽  
Systemic Amyloidosis ◽  
Amyloid P Component ◽  
Serum Amyloid P ◽  
Serum Amyloid P Component ◽  
Amyloid P

Liver-specific and high-level expression of human serum amyloid P component gene in transgenic mice

1989 ◽  
Vol 10 (5) ◽  
pp. 365-371 ◽  
Author(s):  
Tomohisa Iwanaga ◽  
Shoji Wakasugi ◽  
Takeaki Inomoto ◽  
Masahiro Uehira ◽  
Shuji Ohnishi ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Human Serum ◽  
Serum Amyloid ◽  
High Level Expression ◽  
Amyloid P Component ◽  
Serum Amyloid P ◽  
Serum Amyloid P Component ◽  
High Level ◽  
Amyloid P ◽  
Component Gene

scholarly journals Repeat doses of antibody to serum amyloid P component clear amyloid deposits in patients with systemic amyloidosis

2018 ◽  
Vol 10 (422) ◽  
pp. eaan3128 ◽  
Author(s):  
Duncan B. Richards ◽  
Louise M. Cookson ◽  
Sharon V. Barton ◽  
Lia Liefaard ◽  
Thirusha Lane ◽  
...  
Keyword(s):  
Extracellular Volume ◽  
Serum Amyloid ◽  
Systemic Amyloidosis ◽  
Whole Body ◽  
Amyloid P Component ◽  
Serum Amyloid P ◽  
Serum Amyloid P Component ◽  
Amyloid Deposits ◽  
Amyloid Load ◽  
Amyloid P

Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This residual SAP is a specific target for dezamizumab, a fully humanized monoclonal IgG1 anti-SAP antibody that triggers immunotherapeutic clearance of amyloid. We report the safety, pharmacokinetics, and dose-response effects of up to three cycles of miridesap followed by dezamizumab in 23 adult subjects with systemic amyloidosis (ClinicalTrials.gov identifier:NCT01777243). Amyloid load was measured scintigraphically by amyloid-specific radioligand binding of123I-labeled SAP or of99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid. Organ extracellular volume was measured by equilibrium magnetic resonance imaging and liver stiffness by transient elastography. The treatment was well tolerated with the main adverse event being self-limiting early onset rashes after higher antibody doses related to whole body amyloid load. Progressive dose-related clearance of hepatic amyloid was associated with improved liver function tests.123I-SAP scintigraphy confirmed amyloid removal from the spleen and kidneys. No adverse cardiac events attributable to the intervention occurred in the six subjects with cardiac amyloidosis. Amyloid load reduction by miridesap treatment followed by dezamizumab has the potential to improve management and outcome in systemic amyloidosis.

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