Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

Q02985 (FHR3_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 122. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Complement factor H-related protein 3

Short name=FHR-3
Alternative name(s):
DOWN16
H factor-like protein 3
Gene names
Name:CFHR3
Synonyms:CFHL3, FHR3
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length330 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Might be involved in complement regulation.

Subcellular location

Secreted.

Tissue specificity

Expressed by the liver and secreted in plasma.

Sequence similarities

Contains 5 Sushi (CCP/SCR) domains.

Ontologies

Keywords
   Cellular componentSecreted
   Coding sequence diversityAlternative splicing
Polymorphism
   DomainRepeat
Signal
Sushi
   PTMDisulfide bond
Glycoprotein
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Cellular_componentblood microparticle

Inferred from direct assay PubMed 22516433. Source: UniProt

extracellular space

Traceable author statement Ref.1. Source: ProtInc

Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q02985-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
Isoform 2 (identifier: Q02985-2)

The sequence of this isoform differs from the canonical sequence as follows:
     144-204: Missing.
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Signal peptide1 – 1818 Potential
Chain19 – 330312Complement factor H-related protein 3
PRO_0000005898

Regions

Domain22 – 8463Sushi 1
Domain85 – 14258Sushi 2
Domain144 – 20562Sushi 3
Domain208 – 26659Sushi 4
Domain267 – 33064Sushi 5

Amino acid modifications

Glycosylation1081N-linked (GlcNAc...) Ref.7
Glycosylation1851N-linked (GlcNAc...) Potential
Glycosylation2051N-linked (GlcNAc...) Ref.7
Glycosylation3091N-linked (GlcNAc...) Ref.7
Disulfide bond23 ↔ 72 By similarity
Disulfide bond55 ↔ 83 By similarity
Disulfide bond87 ↔ 129 By similarity
Disulfide bond114 ↔ 140 By similarity
Disulfide bond146 ↔ 192 By similarity
Disulfide bond175 ↔ 203 By similarity
Disulfide bond210 ↔ 253 By similarity
Disulfide bond239 ↔ 264 By similarity
Disulfide bond268 ↔ 319 By similarity
Disulfide bond302 ↔ 329 By similarity

Natural variations

Alternative sequence144 – 20461Missing in isoform 2.
VSP_043041
Natural variant711H → Y.
Corresponds to variant rs17575274 [ dbSNP | Ensembl ].
VAR_048817

Experimental info

Sequence conflict1231A → VR in CAA48639. Ref.1
Sequence conflict1321K → N in CAA48639. Ref.1
Sequence conflict1431V → D in CAA48639. Ref.1
Sequence conflict1941Q → R in CAA48639. Ref.1
Sequence conflict2411P → S in CAA48639. Ref.1
Sequence conflict2601E → A in CAA48639. Ref.1

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified February 21, 2001. Version 2.
Checksum: 67B64121D71CD65F

FASTA33037,323
        10         20         30         40         50         60 
MLLLINVILT LWVSCANGQV KPCDFPDIKH GGLFHENMRR PYFPVAVGKY YSYYCDEHFE 

        70         80         90        100        110        120 
TPSGSYWDYI HCTQNGWSPA VPCLRKCYFP YLENGYNQNY GRKFVQGNST EVACHPGYGL 

       130        140        150        160        170        180 
PKAQTTVTCT EKGWSPTPRC IRVRTCSKSD IEIENGFISE SSSIYILNKE IQYKCKPGYA 

       190        200        210        220        230        240 
TADGNSSGSI TCLQNGWSAQ PICINSSEKC GPPPPISNGD TTSFLLKVYV PQSRVEYQCQ 

       250        260        270        280        290        300 
PYYELQGSNY VTCSNGEWSE PPRCIHPCII TEENMNKNNI KLKGRSDRKY YAKTGDTIEF 

       310        320        330 
MCKLGYNANT SILSFQAVCR EGIVEYPRCE 

« Hide

Isoform 2 [UniParc].

Checksum: 401F2F121B468DFA
Show »

FASTA26930,720

References

« Hide 'large scale' references
[1]"A novel short consensus repeat-containing molecule is related to human complement factor H."
Skerka C., Kuehn S., Guenther K., Lingelbach K., Zipfel P.F.
J. Biol. Chem. 268:2904-2908(1993) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
Tissue: Liver.
[2]"Complement factor H: sequence analysis of 221 kb of human genomic DNA containing the entire fH, fHR-1 and fHR-3 genes."
Male D.A., Ormsby R.J., Ranganathan S., Giannakis E., Gordon D.L.
Mol. Immunol. 37:41-52(2000) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S. expand/collapse author list , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2).
Tissue: Liver.
[4]"The DNA sequence and biological annotation of human chromosome 1."
Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A., Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C., Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K. expand/collapse author list , Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C., Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W., Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J., Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J., Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y., Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J., Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H., Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L., Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J., Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S., Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K., Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R., Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M., Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S., Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J., Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W., McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N., Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V., Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J., Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E., Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S., Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M., White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H., Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E., Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G., Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R.
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
Tissue: Liver.
[6]"Complement factor H and related proteins: an expanding family of complement-regulatory proteins?"
Zipfel P.F., Skerka C.
Immunol. Today 15:121-126(1994) [PubMed] [Europe PMC] [Abstract]
Cited for: REVIEW.
[7]"Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.
J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-108; ASN-205 AND ASN-309.
Tissue: Liver.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
X68679 mRNA. Translation: CAA48639.1.
AK298459 mRNA. Translation: BAG60672.1.
AL049741 Genomic DNA. Translation: CAB53064.1.
BC058009 mRNA. Translation: AAH58009.1.
CCDSCCDS30958.1. [Q02985-1]
CCDS53453.1. [Q02985-2]
PIRA45222.
RefSeqNP_001160096.1. NM_001166624.1. [Q02985-2]
NP_066303.2. NM_021023.5. [Q02985-1]
UniGeneHs.709217.

3D structure databases

ProteinModelPortalQ02985.
SMRQ02985. Positions 20-330.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid116086. 2 interactions.
IntActQ02985. 1 interaction.
STRING9606.ENSP00000356395.

Polymorphism databases

DMDM13124752.

Proteomic databases

PaxDbQ02985.
PeptideAtlasQ02985.
PRIDEQ02985.

Protocols and materials databases

DNASU10878.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000367425; ENSP00000356395; ENSG00000116785. [Q02985-1]
ENST00000391985; ENSP00000375845; ENSG00000116785. [Q02985-2]
GeneID10878.
KEGGhsa:10878.
UCSCuc001gtl.3. human. [Q02985-1]
uc010poy.2. human. [Q02985-2]

Organism-specific databases

CTD10878.
GeneCardsGC01P196743.
GeneReviewsCFHR3.
HGNCHGNC:16980. CFHR3.
MIM605336. gene.
neXtProtNX_Q02985.
Orphanet93581. Atypical hemolytic uremic syndrome with anti-factor H antibodies.
PharmGKBPA134909646.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG146710.
HOGENOMHOG000049040.
HOVERGENHBG005665.
InParanoidQ02985.
OMACIRINST.
OrthoDBEOG75XGK1.
PhylomeDBQ02985.
TreeFamTF326157.

Enzyme and pathway databases

ReactomeREACT_6900. Immune System.

Gene expression databases

ArrayExpressQ02985.
BgeeQ02985.
CleanExHS_CFHR3.
GenevestigatorQ02985.

Family and domain databases

InterProIPR000436. Sushi_SCR_CCP.
[Graphical view]
PfamPF00084. Sushi. 4 hits.
[Graphical view]
SMARTSM00032. CCP. 4 hits.
[Graphical view]
SUPFAMSSF57535. SSF57535. 5 hits.
PROSITEPS50923. SUSHI. 3 hits.
[Graphical view]
ProtoNetSearch...

Other

GeneWikiCFHR3.
GenomeRNAi10878.
NextBio41307.
PROQ02985.
SOURCESearch...

Entry information

Entry nameFHR3_HUMAN
AccessionPrimary (citable) accession number: Q02985
Secondary accession number(s): B4DPR0, Q9UJ16
Entry history
Integrated into UniProtKB/Swiss-Prot: June 1, 1994
Last sequence update: February 21, 2001
Last modified: July 9, 2014
This is version 122 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 1

Human chromosome 1: entries, gene names and cross-references to MIM