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Aliases for PRG4 Gene

Aliases for PRG4 Gene

  • Proteoglycan 4 2 3 5
  • Lubricin 2 3 4
  • Articular Superficial Zone Protein 2 3
  • Megakaryocyte Stimulating Factor 2 3
  • Superficial Zone Proteoglycan 3 4
  • MSF 3 4
  • SZP 3 4
  • Proteoglycan 4, (Megakaryocyte Stimulating Factor, Articular Superficial Zone Protein, Camptodactyly, Arthropathy, Coxa Vara, Pericarditis Syndrome) 2
  • Jacobs Camptodactyly-Arthropathy-Pericarditis Syndrome 2
  • BG174L6.2 (MSF: Megakaryocyte Stimulating Factor ) 2
  • Megakaryocyte-Stimulating Factor 4
  • Pericarditis Syndrome 2
  • Hemangiopoietin 3
  • Camptodactyly 2
  • Arthropathy 2
  • Coxa Vara 2
  • HAPO 3
  • JCAP 3
  • CACP 3

External Ids for PRG4 Gene

Previous HGNC Symbols for PRG4 Gene

  • CACP

Previous GeneCards Identifiers for PRG4 Gene

  • GC01P183791
  • GC01P181693
  • GC01P182785
  • GC01P183504
  • GC01P182997
  • GC01P184532
  • GC01P157499

Summaries for PRG4 Gene

Entrez Gene Summary for PRG4 Gene

  • The protein encoded by this gene is a large proteoglycan that is synthesized by chondrocytes located at the surface of articular cartilage and by some synovial lining cells. This protein contains both chondroitin sulfate and keratan sulfate glycosaminoglycans. It functions as a boundary lubricant at the cartilage surface and contributes to the elastic absorption and energy dissipation of synovial fluid. Mutations in this gene result in camptodactyly-arthropathy-coxa vara-pericarditis syndrome. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2014]

GeneCards Summary for PRG4 Gene

PRG4 (Proteoglycan 4) is a Protein Coding gene. Diseases associated with PRG4 include Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome and Pericarditis. Among its related pathways are ERK Signaling and Phospholipase-C Pathway. GO annotations related to this gene include scavenger receptor activity and polysaccharide binding. An important paralog of this gene is ENSG00000273171.

UniProtKB/Swiss-Prot for PRG4 Gene

  • Plays a role in boundary lubrication within articulating joints. Prevents protein deposition onto cartilage from synovial fluid by controlling adhesion-dependent synovial growth and inhibiting the adhesion of synovial cells to the cartilage surface.

  • Isoform F plays a role as a growth factor acting on the primitive cells of both hematopoietic and endothelial cell lineages.

Gene Wiki entry for PRG4 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for PRG4 Gene

Genomics for PRG4 Gene

Regulatory Elements for PRG4 Gene

Enhancers for PRG4 Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around PRG4 on UCSC Golden Path with GeneCards custom track

Genomic Location for PRG4 Gene

186,296,273 bp from pter
186,314,562 bp from pter
18,290 bases
Plus strand

Genomic View for PRG4 Gene

Genes around PRG4 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
PRG4 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for PRG4 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for PRG4 Gene

Proteins for PRG4 Gene

  • Protein details for PRG4 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Proteoglycan 4
    Protein Accession:
    Secondary Accessions:
    • Q6DNC4
    • Q6DNC5
    • Q6ZMZ5
    • Q9BX49

    Protein attributes for PRG4 Gene

    1404 amino acids
    Molecular mass:
    151077 Da
    Quaternary structure:
    • Homodimer; disulfide-linked.
    • Different forms varying in molecular weight have been observed. Such forms are possibly due to different levels of glycosylation and protein cleavage (By similarity).

    Alternative splice isoforms for PRG4 Gene

neXtProt entry for PRG4 Gene

Proteomics data for PRG4 Gene at MOPED

Post-translational modifications for PRG4 Gene

  • N-glycosylated (PubMed:16335952).
  • O-glycosylated; contains glycosaminoglycan chondroitin sulfate and keratan sulfate. O-glycosylated with sialylated oligosaccharides which are predominantly represented by the monosialylated core type I structure, NeuNAcalpha2-3Galbeta1-3GalNAc, with smaller amounts of disialylated O-glycans (PubMed:25187573).
  • The disulfide bond between Cys-1146 and Cys-1403 is essential for protein cleavage.
  • Glycosylation at Ser 123, Ser 136, Asn 206, Thr 240, Thr 253, Thr 277, Thr 291, Thr 305, Ser 306, Thr 310, Ser 317, Thr 324, Thr 332, Thr 338, Thr 367, Thr 376, Thr 384, Thr 385, Ser 388, Thr 391, Thr 399, Thr 407, Thr 408, Thr 415, Thr 423, Ser 427, Thr 430, Thr 438, Thr 439, Thr 446, Thr 447, Thr 454, Thr 455, Thr 477, Thr 478, Thr 485, Thr 493, Thr 494, Thr 501, Thr 502, Thr 509, Thr 525, Ser 529, Thr 532, Thr 540, Thr 541, Ser 553, Thr 555, Thr 563, Thr 564, Thr 571, Thr 572, Thr 579, Thr 580, Thr 587, Thr 588, Thr 595, Thr 603, Thr 604, Thr 611, Thr 612, Thr 616, Thr 619, Thr 627, Thr 676, Thr 683, Thr 684, Thr 691, Thr 692, Thr 699, Thr 700, Thr 704, Thr 707, Thr 723, Thr 724, Thr 736, Thr 768, Thr 769, Thr 776, Thr 777, Thr 792, Thr 793, Thr 805, Ser 812, Thr 829, Thr 837, Thr 838, Ser 892, Thr 900, Thr 930, Thr 931, Ser 962, Thr 963, Thr 968, Thr 975, Thr 978, Thr 979, Thr 980, Thr 1039, Asn 1159, and Thr 1161
  • Modification sites at PhosphoSitePlus

No data available for DME Specific Peptides for PRG4 Gene

Domains & Families for PRG4 Gene

Suggested Antigen Peptide Sequences for PRG4 Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Contains 2 SMB (somatomedin-B) domains.
  • Contains 2 hemopexin repeats.
  • Contains 2 SMB (somatomedin-B) domains.
  • Contains 2 hemopexin repeats.
genes like me logo Genes that share domains with PRG4: view

No data available for Gene Families for PRG4 Gene

Function for PRG4 Gene

Molecular function for PRG4 Gene

UniProtKB/Swiss-Prot Function:
Plays a role in boundary lubrication within articulating joints. Prevents protein deposition onto cartilage from synovial fluid by controlling adhesion-dependent synovial growth and inhibiting the adhesion of synovial cells to the cartilage surface.
UniProtKB/Swiss-Prot Function:
Isoform F plays a role as a growth factor acting on the primitive cells of both hematopoietic and endothelial cell lineages.
genes like me logo Genes that share phenotypes with PRG4: view

Human Phenotype Ontology for PRG4 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

miRNA for PRG4 Gene

miRTarBase miRNAs that target PRG4

Flow Cytometry Products

No data available for Enzyme Numbers (IUBMB) , Gene Ontology (GO) - Molecular Function , Animal Models , Transcription Factor Targets and HOMER Transcription for PRG4 Gene

Localization for PRG4 Gene

Subcellular locations from UniProtKB/Swiss-Prot for PRG4 Gene


Subcellular locations from

Jensen Localization Image for PRG4 Gene COMPARTMENTS Subcellular localization image for PRG4 gene
Compartment Confidence
extracellular 4
nucleus 3
cytosol 2
endoplasmic reticulum 1
golgi apparatus 1

No data available for Gene Ontology (GO) - Cellular Components for PRG4 Gene

Pathways & Interactions for PRG4 Gene

genes like me logo Genes that share pathways with PRG4: view

Pathways by source for PRG4 Gene

Gene Ontology (GO) - Biological Process for PRG4 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006898 receptor-mediated endocytosis IEA --
GO:0008283 cell proliferation TAS 10545950
genes like me logo Genes that share ontologies with PRG4: view

No data available for SIGNOR curated interactions for PRG4 Gene

Drugs & Compounds for PRG4 Gene

(2) Drugs for PRG4 Gene - From: Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials

(4) Additional Compounds for PRG4 Gene - From: Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
genes like me logo Genes that share compounds with PRG4: view

Transcripts for PRG4 Gene

Unigene Clusters for PRG4 Gene

Proteoglycan 4:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for PRG4 Gene

ExUns: 1 ^ 2 ^ 3 ^ 4 ^ 5a · 5b ^ 6a · 6b ^ 7 ^ 8 ^ 9 ^ 10 ^ 11 ^ 12 ^ 13
SP3: -
SP4: - -

Relevant External Links for PRG4 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for PRG4 Gene

mRNA expression in normal human tissues for PRG4 Gene

mRNA differential expression in normal tissues according to GTEx for PRG4 Gene

This gene is overexpressed in Liver (x14.2), Adipose - Visceral (Omentum) (x8.5), Heart - Atrial Appendage (x5.0), and Adipose - Subcutaneous (x4.9).

Protein differential expression in normal tissues from HIPED for PRG4 Gene

This gene is overexpressed in Synovial fluid (27.2), Serum (16.7), Monocytes (14.7), and Plasma (6.3).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, and MOPED for PRG4 Gene

SOURCE GeneReport for Unigene cluster for PRG4 Gene Hs.647723

mRNA Expression by UniProt/SwissProt for PRG4 Gene

Tissue specificity: Highly expressed in synovial tissue, cartilage and liver and weakly in heart and lung. Isoform B is expressed in kidney, lung, liver, heart and brain. Isoform C and isoform D are widely expressed.
genes like me logo Genes that share expression patterns with PRG4: view

Primer Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and Protein tissue co-expression partners for PRG4 Gene

Orthologs for PRG4 Gene

This gene was present in the common ancestor of chordates.

Orthologs for PRG4 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia PRG4 35
  • 81.51 (n)
  • 74.19 (a)
PRG4 36
  • 68 (a)
(Pan troglodytes)
Mammalia PRG4 35
  • 96.8 (n)
  • 95.68 (a)
(Rattus norvegicus)
Mammalia Prg4 35
  • 79.21 (n)
  • 76.01 (a)
(Canis familiaris)
Mammalia PRG4 36
  • 77 (a)
(Monodelphis domestica)
Mammalia PRG4 36
  • 50 (a)
(Mus musculus)
Mammalia Prg4 36
  • 68 (a)
(Ornithorhynchus anatinus)
Mammalia PRG4 36
  • 40 (a)
(Gallus gallus)
Aves PRG4 36
  • 34 (a)
(Anolis carolinensis)
Reptilia PRG4 36
  • 29 (a)
(Danio rerio)
Actinopterygii prg4a 36
  • 33 (a)
prg4b 36
  • 32 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 20 (a)
Species with no ortholog for PRG4:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • tropical clawed frog (Silurana tropicalis)
  • wheat (Triticum aestivum)
  • worm (Caenorhabditis elegans)

Evolution for PRG4 Gene

Gene Tree for PRG4 (if available)
Gene Tree for PRG4 (if available)

Paralogs for PRG4 Gene

Paralogs for PRG4 Gene

(1) SIMAP similar genes for PRG4 Gene using alignment to 4 proteins:

genes like me logo Genes that share paralogs with PRG4: view

Variants for PRG4 Gene

Sequence variations from dbSNP and Humsavar for PRG4 Gene

SNP ID Clin Chr 01 pos Sequence Context AA Info Type
rs2273779 - 186,304,862(-) TTTCC(A/G)AATTG intron-variant, reference, missense
rs10158395 - 186,309,108(+) ACCCA(A/G)TCAAG reference, missense
VAR_051560 -
VAR_051561 -
rs1293987 -- 186,296,816(+) AGAGC(A/T)GTTTT intron-variant

Variation tolerance for PRG4 Gene

Residual Variation Intolerance Score: 93.4% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 10.96; 91.98% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for PRG4 Gene

Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot and Structural Variations from Database of Genomic Variants (DGV) for PRG4 Gene

Disorders for PRG4 Gene

MalaCards: The human disease database

(6) MalaCards diseases for PRG4 Gene - From: OMIM, ClinVar, GeneTests, Orphanet, DISEASES, Novoseek, and GeneCards

Disorder Aliases PubMed IDs
camptodactyly-arthropathy-coxa vara-pericarditis syndrome
  • cdags syndrome
  • ankylosis of ankle and foot joint
pericardium disease
- elite association - COSMIC cancer census association via MalaCards
Search PRG4 in MalaCards View complete list of genes associated with diseases


  • Camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) [MIM:208250]: An autosomal recessive disorder characterized by the association of congenital or early-onset camptodactyly and non-inflammatory arthropathy with synovial hyperplasia. Individuals with CACP have normal appearing joints at birth but with advancing age develop joint failure, non-inflammatory synoviocyte hyperplasia and subintimal fibrosis of the synovial capsule. Some patients also manifest progressive coxa vara deformity and/or non-inflammatory pericardial or pleural effusions. {ECO:0000269 PubMed:10545950}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Relevant External Links for PRG4

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with PRG4: view

No data available for Genatlas for PRG4 Gene

Publications for PRG4 Gene

  1. Articular cartilage superficial zone protein (SZP) is homologous to megakaryocyte stimulating factor precursor and is a multifunctional proteoglycan with potential growth-promoting, cytoprotective, and lubricating properties in cartilage metabolism. (PMID: 9920774) Flannery C.R. … Caterson B. (Biochem. Biophys. Res. Commun. 1999) 2 3 4 23 67
  2. CACP, encoding a secreted proteoglycan, is mutated in camptodactyly- arthropathy-coxa vara-pericarditis syndrome. (PMID: 10545950) Marcelino J. … Warman M.L. (Nat. Genet. 1999) 2 3 23
  3. Boundary mode lubrication of articular cartilage by recombinant human lubricin. (PMID: 19058183) Gleghorn J.P. … Bonassar L.J. (J. Orthop. Res. 2009) 3 23
  4. Decreased lubricin concentrations and markers of joint inflammation in the synovial fluid of patients with anterior cruciate ligament injury. (PMID: 18512776) Elsaid K.A. … Jay G.D. (Arthritis Rheum. 2008) 3 23
  5. Cartilaginous deposits in subchondral bone in regions of exposed bone in osteoarthritis of the human knee: histomorphometric study of PRG4 distribution in osteoarthritic cartilage. (PMID: 17343281) Zhang D. … Spector M. (J. Orthop. Res. 2007) 3 23

Products for PRG4 Gene

Sources for PRG4 Gene