Serum amyloid P component
The serum amyloid P component
(SAP) is the identical serum form of
amyloid P component (AP), a 25kDa
pentameric protein first identified as
the pentagonal constituent of in vivo
pathological deposits called
"amyloid".[5] APCS is its human
gene.[6]
In amyloidosis
AP makes up 14% of the dry mass of
amyloid deposits[7] and is thought to
be an important contributor to the
pathogenesis of a related group of
diseases called the Amyloidoses.[8]
These conditions are characterised by
the ordered aggregation of normal
globular proteins and peptides into
insoluble fibres which disrupt tissue
architecture and are associated with
cell death. AP is thought to decorate
and stabilise aggregates by
preventing proteolytic cleavage and
hence inhibiting fibril removal via the
normal protein scavenging
mechanisms. [9] This association is
utilised in the routine clinical
diagnostic technique of SAP
scintigraphy whereby radio-labelled
protein is injected into patients to
locate areas of amyloid
deposition.[10] The SAP-amyloid
association has also been identified
as a possible drug target for anti-
amyloid therapy, with the recent
development and first stage clinical
trials of a compound called CPHPC
(R-1-[6-[R-2-carboxy-pyrrolidin-1-
yl]-6-oxohexanoyl] pyrrolidine-2-
carboxylic acid), a small molecule
able to strip AP from deposits by
reducing levels of circulating
SAP.[11]
APCS
Available structures
PDB Ortholog search: PDBe (https://www.ebi.ac.uk/pdbe/search
Results.html?display=both&term=P12246%20or%20P0274
3) RCSB (http://www.rcsb.org/pdb/search/smartSubquery.d
o?smartSearchSubtype=UpAccessionIdQuery&accessionId
List=P12246,P02743)
List of PDB id codes
4AYU (https://www.rcsb.org/structure/4AYU), 1GYK
(https://www.rcsb.org/structure/1GYK), 1LGN (https://
www.rcsb.org/structure/1LGN), 1SAC (https://www.rc
sb.org/structure/1SAC), 2A3W (https://www.rcsb.org/
structure/2A3W), 2A3X (https://www.rcsb.org/structur
e/2A3X), 2A3Y
(https://www.rcsb.org/structure/2A3Y), 2W08 (https://
www.rcsb.org/structure/2W08), 3D5O (https://www.rc
sb.org/structure/3D5O), 3KQR (https://www.rcsb.org/
structure/3KQR), 4AVS (https://www.rcsb.org/structur
e/4AVS), 4AVT (https://www.rcsb.org/structure/4AV
T), 4AVV (https://www.rcsb.org/structure/4AVV)
Identifiers
Aliases APCS (https://www.genenames.org/data/gene-symbol-re
port/#!/hgnc_id/584), HEL-S-92n, PTX2, SAP, amyloid P
component, serum
External OMIM: 104770 (https://omim.org/entry/104770) MGI:
IDs 98229 (http://www.informatics.jax.org/marker/MGI:98229)
HomoloGene: 123932 (https://www.ncbi.nlm.nih.gov/entr
ez/query.fcgi?cmd=Retrieve&db=homologene&dopt=Hom
oloGene&list_uids=123932) GeneCards: APCS (https://w
ww.genecards.org/cgi-bin/carddisp.pl?gene=APCS)
 Gene location (Human)
Structure
SAP is a member of the pentraxins
family, characterised by calcium
dependent ligand binding and
distinctive flattened β-jellyroll
structure similar to that of the legume
lectins.[12] The name "pentraxin" is Chr. Chromosome 1 (human)[1]
derived from the Greek word for five
(penta) and berries (ragos) relating to
the radial symmetry of five
monomers forming a ring
approximately 95 Å across and 35 Å Band 1q23.2 Start 159,587,826 bp[1]
deep. Human SAP has 51% End 159,588,865 bp[1]
sequence homology with C-reactive
protein (CRP), a classical acute Gene location (Mouse)
phase response plasma protein, and is
a more distant relative to the "long"
pentraxins such as PTX3 (a cytokine
modulated molecule) and several
neuronal pentraxins. Both SAP and
CRP are evolutionary conserved in
all vertebrates and also found in Chr. Chromosome 1 (mouse)[2]
distant invertebrates such as the
horseshoe crab (Limulus
polyphemus). [13]

Band 1 H3|1 80.33 cM Start 172,893,961 bp[2]

References End 172,895,041 bp[2]

1. GRCh38: Ensembl release RNA expression pattern

89: ENSG00000132703 (htt
p://May2017.archive.ensemb
l.org/Homo_sapiens/Gene/Su
mmary?db=core;g=ENSG000
00132703) - Ensembl, May
2017
2. GRCm38: Ensembl release
89: ENSMUSG00000026542
(http://May2017.archive.ense
mbl.org/Mus_musculus/Gene/
Summary?db=core;g=ENSM More reference expression data (http://biogps.org/gene/325/)
USG00000026542) -
Ensembl, May 2017 Gene ontology
3. "Human PubMed Reference:" Molecular • calcium ion binding (http://amigo.geneontology.org/a
(https://www.ncbi.nlm.nih.gov/
function migo/term/GO:0005509)
sites/entrez?db=gene&cmd=
Link&LinkName=gene_pubm • unfolded protein binding (http://amigo.geneontology.
ed&from_uid=325). National org/amigo/term/GO:0051082)
Center for Biotechnology • metal ion binding (http://amigo.geneontology.org/ami
Information, U.S. National go/term/GO:0046872)
Library of Medicine.
• virion binding (http://amigo.geneontology.org/amigo/t
4. "Mouse PubMed Reference:"
(https://www.ncbi.nlm.nih.gov/
sites/entrez?db=gene&cmd=
Link&LinkName=gene_pubm
ed&from_uid=20219).
National Center for
Biotechnology Information,
U.S. National Library of
Medicine.
5. Cathcart ES; Shirahama T;
Cohen AS (1967). "Isolation
and identification of a plasma
component of amyloid".
Biochim. Biophys. Acta. 147
(2): 392–393.
doi:10.1016/0005-
2795(67)90420-5 (https://doi.
org/10.1016%2F0005-2795%
2867%2990420-5).
6. "Entrez Gene: APCS amyloid
P component, serum" (https://
www.ncbi.nlm.nih.gov/sites/e
ntrez?Db=gene&Cmd=Show
DetailView&TermToSearch=3
25).
7. Skinner M; Pepys MB; Cohen
AS; Heller LM; Lian JB
(1980). Freitas, Antonio
Falcão de; Glenner, George
G.; Costa, Pedro Pinho e.
(eds.). Amyloid and
amyloidosis: proceedings of
the Third International
Symposium on Amyloidosis,
Póvoa de Varzim, Portugal,
23–28 September 1979.
Amsterdam: Excerpta
Medica. pp. 384–391.
ISBN 0-444-90124-8.
8. Botto M, Hawkins PN,
Bickerstaff MC, Herbert J,
Bygrave AE, McBride A,
Hutchinson WL, Tennent GA,
Walport MJ, Pepys MB
(August 1997). "Amyloid
deposition is delayed in mice
with targeted deletion of the
serum amyloid P component
gene". Nature Medicine. 3 (8):
855–9. doi:10.1038/9544 (htt
ps://doi.org/10.1038%2F954
4). PMID 9256275 (https://pub
med.ncbi.nlm.nih.gov/925627
5).
erm/GO:0046790)
• complement component C1q binding (http://amigo.g
eneontology.org/amigo/term/GO:0001849)
• carbohydrate binding (http://amigo.geneontology.or
g/amigo/term/GO:0030246)
• identical protein binding (http://amigo.geneontology.
org/amigo/term/GO:0042802)
• low-density lipoprotein particle binding (http://amigo.
geneontology.org/amigo/term/GO:0030169)
Cellular • blood microparticle (http://amigo.geneontology.org/a
component migo/term/GO:0072562)
• extracellular matrix (http://amigo.geneontology.org/a
migo/term/GO:0031012)
• extracellular exosome (http://amigo.geneontology.or
g/amigo/term/GO:0070062)
• cell nucleus (http://amigo.geneontology.org/amigo/te
rm/GO:0005634)
• extracellular (http://amigo.geneontology.org/amigo/te
rm/GO:0005615)
• extracellular region (http://amigo.geneontology.org/a
migo/term/GO:0005576)
• collagen-containing extracellular matrix (http://amig
o.geneontology.org/amigo/term/GO:0062023)
Biological • cellular protein metabolic process (http://amigo.gene
process ontology.org/amigo/term/GO:0044267)
• negative regulation of monocyte differentiation (htt
p://amigo.geneontology.org/amigo/term/GO:0045656)
• negative regulation of acute inflammatory response
(http://amigo.geneontology.org/amigo/term/GO:00026
74)
• negative regulation of viral entry into host cell (http://
amigo.geneontology.org/amigo/term/GO:0046597)
• chaperone-mediated protein complex assembly (htt
p://amigo.geneontology.org/amigo/term/GO:0051131)
• negative regulation of viral process (http://amigo.gen
eontology.org/amigo/term/GO:0048525)
• negative regulation of glycoprotein metabolic
process (http://amigo.geneontology.org/amigo/term/G
O:1903019)
• protein folding (http://amigo.geneontology.org/amig
o/term/GO:0006457)
• acute-phase response (http://amigo.geneontology.or
g/amigo/term/GO:0006953)
• negative regulation of exo-alpha-sialidase activity (ht
tp://amigo.geneontology.org/amigo/term/GO:1903016)
• negative regulation of wound healing (http://amigo.g
 9. Tennent GA, Lovat LB, Pepys
MB (May 1995). "Serum
amyloid P component
prevents proteolysis of the
amyloid fibrils of Alzheimer
disease and systemic
amyloidosis" (http://www.pna
s.org/cgi/reprint/92/10/4299.p
df) (PDF). Proceedings of the
National Academy of
Sciences of the United States
of America. 92 (10): 4299–
303.
doi:10.1073/pnas.92.10.4299
(https://doi.org/10.1073%2Fp
nas.92.10.4299). PMC 41931
(https://www.ncbi.nlm.nih.gov/
pmc/articles/PMC41931).
PMID 7753801 (https://pubme
d.ncbi.nlm.nih.gov/7753801).
10. Hawkins PN, Pepys MB (July
1995). "Imaging amyloidosis
with radiolabelled SAP".
European Journal of Nuclear
Medicine. 22 (7): 595–9.
doi:10.1007/BF01254559 (htt
ps://doi.org/10.1007%2FBF0
1254559). PMID 7498219 (htt
ps://pubmed.ncbi.nlm.nih.gov/
7498219).
11. Pepys MB, Herbert J,
Hutchinson WL, Tennent GA,
Lachmann HJ, Gallimore JR,
Lovat LB, Bartfai T, Alanine A,
Hertel C, Hoffmann T, Jakob-
Roetne R, Norcross RD,
Kemp JA, Yamamura K,
Suzuki M, Taylor GW, Murray
S, Thompson D, Purvis A,
Kolstoe S, Wood SP,
Hawkins PN (May 2002).
"Targeted pharmacological
depletion of serum amyloid P
component for treatment of
human amyloidosis". Nature.
417 (6886): 254–9.
doi:10.1038/417254a (https://
doi.org/10.1038%2F417254
a). PMID 12015594 (https://pu
bmed.ncbi.nlm.nih.gov/12015
594).
eneontology.org/amigo/term/GO:0061045)
• innate immune system (http://amigo.geneontology.or
g/amigo/term/GO:0045087)
• Classical complement pathway (http://amigo.geneon
tology.org/amigo/term/GO:0006958)
Sources:Amigo (http://amigo.geneontology.org/) / QuickGO (https://
www.ebi.ac.uk/QuickGO/)
Orthologs
Species Human Mouse
Entrez
325 (https://www.ncbi. 20219 (https://www.ncbi.
nlm.nih.gov/entrez/qu nlm.nih.gov/entrez/query.f
ery.fcgi?db=gene&cm cgi?db=gene&cmd=retrie
d=retrieve&dopt=defa ve&dopt=default&list_uid
ult&list_uids=325&rn= s=20219&rn=1)
1)
Ensembl
ENSG00000132703 ENSMUSG00000026542
(http://www.ensembl.o (http://www.ensembl.org/
rg/Homo_sapiens/gen Mus_musculus/genevie
eview?gene=ENSG00 w?gene=ENSMUSG000
000132703;db=core) 00026542;db=core)
UniProt
P02743 (https://www.u P12246 (https://www.unip
niprot.org/uniprot/P02 rot.org/uniprot/P12246)
743)
RefSeq
(mRNA) NM_001639 (https://w NM_011318 (https://ww
ww.ncbi.nlm.nih.gov/e w.ncbi.nlm.nih.gov/entre
ntrez/viewer.fcgi?val= z/viewer.fcgi?val=NM_01
NM_001639) 1318)
RefSeq
(protein) NP_001630 (https://w NP_035448 (https://www.
ww.ncbi.nlm.nih.gov/e ncbi.nlm.nih.gov/entrez/vi
ntrez/viewer.fcgi?val= ewer.fcgi?val=NP_03544
NP_001630) 8)
Location Chr 1: 159.59 – 159.59 Chr 1: 172.89 – 172.9 Mb (ht
(UCSC) Mb (https://genome.ucsc. tps://genome.ucsc.edu/cgi-bi
edu/cgi-bin/hgTracks?org n/hgTracks?org=Mouse&db=
=Human&db=hg38&positi mm0&position=chr1:1728939
on=chr1:159587826-1595 61-172895041)
88865)
 [3] [4]
12. Emsley J, White HE, O'Hara PubMed
BP, Oliva G, Srinivasan N, search
Tickle IJ, Blundell TL, Pepys
Wikidata
MB, Wood SP (January
1994). "Structure of View/Edit Human View/Edit Mouse
pentameric human serum
amyloid P component".
Nature. 367 (6461): 338–45.
doi:10.1038/367338a0 (http
s://doi.org/10.1038%2F36733
8a0). PMID 8114934 (https://p
ubmed.ncbi.nlm.nih.gov/8114
934).
13. Pepys MB, Booth DR,
Hutchinson WL, Gallimore
JR, Collins PM, Hohenester
E (1997). "Amyloid P
component. A critical review".
Amyloid. 4 (4): 274–295.
doi:10.3109/1350612970900
3838 (https://doi.org/10.310
9%2F13506129709003838).

Retrieved from "https://en.wikipedia.org/w/index.php?title=Serum_amyloid_P_component&oldid=946439951"

This page was last edited on 20 March 2020, at 04:31 (UTC).

Text is available under the Creative Commons Attribution-ShareAlike License; additional terms may apply. By using this
site, you agree to the Terms of Use and Privacy Policy. Wikipedia® is a registered trademark of the Wikimedia
Foundation, Inc., a non-profit organization.