Gene Symbol: Matn1
Description: matrilin 1, cartilage matrix protein
Alias: CMP, Crtm, Mat1, matrilin-1, cartilage matrix protein, matrilin 1, cartilage matrix protein 1
Species: mouse
Products:     Matn1

Top Publications

  1. Aszodi A, Bateman J, Hirsch E, Baranyi M, Hunziker E, Hauser N, et al. Normal skeletal development of mice lacking matrilin 1: redundant function of matrilins in cartilage?. Mol Cell Biol. 1999;19:7841-5 pubmed
    Matrilin 1, or cartilage matrix protein, is a member of a novel family of extracellular matrix proteins. To date, four members of the family have been identified, but their biological role is unknown...
  2. Nicolae C, Ko Y, Miosge N, Niehoff A, Studer D, Enggist L, et al. Abnormal collagen fibrils in cartilage of matrilin-1/matrilin-3-deficient mice. J Biol Chem. 2007;282:22163-75 pubmed
    ..Surprisingly, however, Matn3-null as well as Matn1- and Matn2-null mice do not show an overt skeletal phenotype, suggesting a dominant negative pathomechanism for ..
  3. Hyde G, Dover S, Aszodi A, Wallis G, Boot Handford R. Lineage tracing using matrilin-1 gene expression reveals that articular chondrocytes exist as the joint interzone forms. Dev Biol. 2007;304:825-33 pubmed
    We have developed a mouse in which the Cre recombinase gene has been targeted to exon 1 of the matrilin-1 gene (Matn1) to investigate the origins of articular chondrocytes and the development of the knee joint...
  4. Smits P, Li P, Mandel J, Zhang Z, Deng J, Behringer R, et al. The transcription factors L-Sox5 and Sox6 are essential for cartilage formation. Dev Cell. 2001;1:277-90 pubmed
    ..L-Sox5 and Sox6 are thus redundant, potent enhancers of chondroblast functions, thereby essential for endochondral skeleton formation. ..
  5. Lawson K, Teteak C, Zou J, Hacquebord J, Ghatan A, Zielinska Kwiatkowska A, et al. Mesenchyme-specific knockout of ESET histone methyltransferase causes ectopic hypertrophy and terminal differentiation of articular chondrocytes. J Biol Chem. 2013;288:32119-25 pubmed publisher
  6. Chen M, Doostan A, Bandyopadhyay P, Remington J, Wang X, Hou Y, et al. The cartilage matrix protein subdomain of type VII collagen is pathogenic for epidermolysis bullosa acquisita. Am J Pathol. 2007;170:2009-18 pubmed
    ..domain corresponding to the N-terminal 227 amino acids (residues 1 to 227), which contains homology with cartilage matrix protein (CMP)...
  7. Feenstra J, Kanaya K, Pira C, Hoffman S, Eppey R, Oberg K. Detection of genes regulated by Lmx1b during limb dorsalization. Dev Growth Differ. 2012;54:451-62 pubmed publisher
    ..Our study provides the most comprehensive characterization of genes regulated by Lmx1b during limb development to-date and provides targets for further investigation. ..
  8. Aszodi A, Hunziker E, Olsen B, Fassler R. The role of collagen II and cartilage fibril-associated molecules in skeletal development. Osteoarthritis Cartilage. 2001;9 Suppl A:S150-9 pubmed
    ..Collagen II is important for cartilage formation, collagen IX for cartilage maintenance and matrilin-1 is redundant. ..
  9. Costell M, Gustafsson E, Aszodi A, Morgelin M, Bloch W, Hunziker E, et al. Perlecan maintains the integrity of cartilage and some basement membranes. J Cell Biol. 1999;147:1109-22 pubmed

More Information


  1. Aszodi A, Chan D, Hunziker E, Bateman J, Fassler R. Collagen II is essential for the removal of the notochord and the formation of intervertebral discs. J Cell Biol. 1998;143:1399-412 pubmed
  2. Paulsson M, Piecha D, Segat D, Smyth N, Wagener R. The matrilins: a growing family of A-domain-containing proteins. Biochem Soc Trans. 1999;27:824-6 pubmed
  3. Deak F, Piecha D, Bachrati C, Paulsson M, Kiss I. Primary structure and expression of matrilin-2, the closest relative of cartilage matrix protein within the von Willebrand factor type A-like module superfamily. J Biol Chem. 1997;272:9268-74 pubmed
    ..A sequence similarity search indicated the closest relation to the trimeric cartilage matrix protein (CMP)...
  4. Decker R, Um H, Dyment N, Cottingham N, Usami Y, Enomoto Iwamoto M, et al. Cell origin, volume and arrangement are drivers of articular cartilage formation, morphogenesis and response to injury in mouse limbs. Dev Biol. 2017;426:56-68 pubmed publisher
    ..Synovial Prg4+ lineage progenitors are exquisitely responsive to acute injury and may represent pioneers in joint tissue repair. ..
  5. Szénási T, Kenesi E, Nagy A, Molnar A, Balint B, Zvara A, et al. Hmgb1 can facilitate activation of the matrilin-1 gene promoter by Sox9 and L-Sox5/Sox6 in early steps of chondrogenesis. Biochim Biophys Acta. 2013;1829:1075-91 pubmed publisher
    ..Here we report its contribution to the evolutionarily conserved, distinctive regulation of the matrilin-1 gene (Matn1) expression in amniotes...
  6. Kahn J, Shwartz Y, Blitz E, Krief S, Sharir A, Breitel D, et al. Muscle contraction is necessary to maintain joint progenitor cell fate. Dev Cell. 2009;16:734-43 pubmed publisher
    ..In conclusion, our findings provide the missing link between progenitor cell fate determination and embryonic movement, two processes shown to be essential for correct organogenesis. ..
  7. Allen J, Bateman J, Hansen U, Wilson R, Bruckner P, Owens R, et al. WARP is a novel multimeric component of the chondrocyte pericellular matrix that interacts with perlecan. J Biol Chem. 2006;281:7341-9 pubmed
    ..We conclude that WARP forms macromolecular structures that interact with perlecan to contribute to the assembly and/or maintenance of "permanent" cartilage structures during development and in mature cartilages. ..
  8. Murphy J, Heinegård R, McIntosh A, Sterchi D, Barry F. Distribution of cartilage molecules in the developing mouse joint. Matrix Biol. 1999;18:487-97 pubmed
    ..of protein distribution for aggrecan, fibromodulin, cartilage oligomeric matrix protein (COMP) and cartilage matrix protein (CMP) in the developing mouse limb with particular attention to those cells destined to form articular ..
  9. Kamper M, Paulsson M, Zaucke F. Absence of collagen IX accelerates hypertrophic differentiation in the embryonic mouse spine through a disturbance of the Ihh-PTHrP feedback loop. Cell Tissue Res. 2017;367:359-367 pubmed publisher
  10. Wai A, Ng L, Watanabe H, Yamada Y, Tam P, Cheah K. Disrupted expression of matrix genes in the growth plate of the mouse cartilage matrix deficiency (cmd) mutant. Dev Genet. 1998;22:349-58 pubmed
    ..The discordance of gene expression in cmd chondrocytes may be additional factors contributing to the disrupted cellular architecture of the growth plate resulting from the primary absence of aggrecan. ..
  11. Mundy C, Yasuda T, Kinumatsu T, Yamaguchi Y, Iwamoto M, Enomoto Iwamoto M, et al. Synovial joint formation requires local Ext1 expression and heparan sulfate production in developing mouse embryo limbs and spine. Dev Biol. 2011;351:70-81 pubmed publisher
    ..The data indicate also that defects in joint formation reverberate on, and delay, overall long bone growth. ..
  12. Huang X, Birk D, Goetinck P. Mice lacking matrilin-1 (cartilage matrix protein) have alterations in type II collagen fibrillogenesis and fibril organization. Dev Dyn. 1999;216:434-41 pubmed
    Matrilin-1 (cartilage matrix protein) is a homotrimeric protein that forms collagen-dependent and collagen-independent fibrils in the extracellular matrix of cartilage...
  13. Aszodi A, Hauser N, Studer D, Paulsson M, Hiripi L, Bosze Z. Cloning, sequencing and expression analysis of mouse cartilage matrix protein cDNA. Eur J Biochem. 1996;236:970-7 pubmed
    A cDNA encoding the mouse cartilage matrix protein (CMP) was cloned following the reverse-transcription polymerase chain reaction and rapid amplification of cDNA ends procedures using mRNA isolated from trachea...
  14. Chen Y, Cossman J, Jayasuriya C, Li X, Guan Y, Fonseca V, et al. Deficient Mechanical Activation of Anabolic Transcripts and Post-Traumatic Cartilage Degeneration in Matrilin-1 Knockout Mice. PLoS ONE. 2016;11:e0156676 pubmed publisher
    Matrilin-1 (Matn1), a cartilage-specific peri-cellular and extracellular matrix (ECM) protein, has been hypothesized to regulate ECM interactions and transmit mechanical signals in cartilage...
  15. Schmitz M, Niehoff A, Miosge N, Smyth N, Paulsson M, Zaucke F. Transgenic mice expressing D469Delta mutated cartilage oligomeric matrix protein (COMP) show growth plate abnormalities and sternal malformations. Matrix Biol. 2008;27:67-85 pubmed
    ..Later in development, the growth plate was irregularly shaped and prematurely invaded by bony tissue. In addition, a fusion of the third and fourth sternebrae was frequently observed. ..
  16. Malin D, Sonnenberg Riethmacher E, Guseva D, Wagener R, Aszodi A, Irintchev A, et al. The extracellular-matrix protein matrilin 2 participates in peripheral nerve regeneration. J Cell Sci. 2009;122:995-1004 pubmed publisher
    ..These results show that matrilin 2 is a permissive substrate for axonal growth and cell migration, and that it is required for successful nerve regeneration. ..
  17. Foradori M, Chen Q, Fernandez C, Harper J, Li X, Tsang P, et al. Matrilin-1 is an inhibitor of neovascularization. J Biol Chem. 2014;289:14301-9 pubmed publisher
  18. Aszodi A, Beier D, Hiripi L, Bosze Z, Fassler R. Sequence, structure and chromosomal localization of Crtm gene encoding mouse cartilage matrix protein and its exclusion as a candidate for murine achondroplasia. Matrix Biol. 1998;16:563-73 pubmed
    The mouse cartilage matrix protein gene (Crtm) was isolated from a cosmid library using a mouse Crtm cDNA fragment as probe. Crtm spans 12.2 kb from the start of translation to the polyadenylation signal sequence and comprises eight exons...
  19. Kawakami Y, Yoshida K, Yang J, Suzuki T, Azuma N, Sakai K, et al. Impaired neurogenesis in embryonic spinal cord of Phgdh knockout mice, a serine deficiency disorder model. Neurosci Res. 2009;63:184-93 pubmed publisher
    ..The present integrative study provides in vivo evidence that normal cell cycle progression and subsequent neurogenesis of radial glia cells are severely impaired by serine deficiency. ..
  20. Jenner F, IJpma A, Cleary M, Heijsman D, Narcisi R, van der Spek P, et al. Differential gene expression of the intermediate and outer interzone layers of developing articular cartilage in murine embryos. Stem Cells Dev. 2014;23:1883-98 pubmed publisher
    ..The high representation of genes relevant to chondrocyte hypertrophy and endochondral ossification in the outer interzone suggests that it undergoes endochondral ossification. ..
  21. Smits P, Dy P, Mitra S, Lefebvre V. Sox5 and Sox6 are needed to develop and maintain source, columnar, and hypertrophic chondrocytes in the cartilage growth plate. J Cell Biol. 2004;164:747-58 pubmed
    ..In conclusion, Sox5 and Sox6 are needed for the establishment of multilayered growth plates, and thereby for proper and timely development of endochondral bones. ..
  22. Segat D, Frie C, Nitsche P, Klatt A, Piecha D, Korpos E, et al. Expression of matrilin-1, -2 and -3 in developing mouse limbs and heart. Matrix Biol. 2000;19:649-55 pubmed
    ..The complementary, but in part overlapping, expression of matrilins indicates the possibility for both redundant and unique functions among the members of this novel family of extracellular matrix proteins. ..
  23. Kanbe K, Yang X, Wei L, Sun C, Chen Q. Pericellular matrilins regulate activation of chondrocytes by cyclic load-induced matrix deformation. J Bone Miner Res. 2007;22:318-28 pubmed
    ..matrilins were decreased by expressing a dominant negative mini-MATN in primary chondrocytes or by using MATN1-null chondrocytes...
  24. Mátés L, Nicolae C, Morgelin M, Deak F, Kiss I, Aszodi A. Mice lacking the extracellular matrix adaptor protein matrilin-2 develop without obvious abnormalities. Matrix Biol. 2004;23:195-204 pubmed
  25. Ko Y, Kobbe B, Nicolae C, Miosge N, Paulsson M, Wagener R, et al. Matrilin-3 is dispensable for mouse skeletal growth and development. Mol Cell Biol. 2004;24:1691-9 pubmed
    ..Altogether, our findings suggest functional redundancy among matrilins and demonstrate that the phenotypes of MED disorders are not caused by the absence of matrilin-3 in cartilage ECM. ..
  26. Ye T, Gordon C, Lai Y, Fujiwara Y, Peters L, Perkins A, et al. Ermap, a gene coding for a novel erythroid specific adhesion/receptor membrane protein. Gene. 2000;242:337-45 pubmed
    ..Northern blot analysis and in-situ hybridization demonstrated that Ermap expression was restricted to fetal and adult erythroid tissues. ERMAP is likely a novel adhesion/receptor molecule specific for erythroid cells. ..
  27. Bell P, Piróg K, Fresquet M, Thornton D, Boot Handford R, Briggs M. Loss of matrilin 1 does not exacerbate the skeletal phenotype in a mouse model of multiple epiphyseal dysplasia caused by a Matn3 V194D mutation. Arthritis Rheum. 2012;64:1529-39 pubmed publisher was proposed that secretion of mutant matrilin 3 may be dependent on the formation of hetero-oligomers with matrilin 1. The aim of this study was to investigate the hypothesis that deletion of matrilin 1 would abolish the formation ..
  28. Klatt A, Nitsche D, Kobbe B, Macht M, Paulsson M, Wagener R. Molecular structure, processing, and tissue distribution of matrilin-4. J Biol Chem. 2001;276:17267-75 pubmed
    ..In the matrix formed by cultured primary embryonic fibroblasts, matrilin-4 is found in a filamentous network connecting individual cells. ..
  29. van der Weyden L, Wei L, Luo J, Yang X, Birk D, Adams D, et al. Functional knockout of the matrilin-3 gene causes premature chondrocyte maturation to hypertrophy and increases bone mineral density and osteoarthritis. Am J Pathol. 2006;169:515-27 pubmed
    ..However, by 18 weeks of age, Matn3 null mice had a significantly higher total body bone mineral density than Matn1 null mice or wild-type littermates...
  30. McDonald K, Fudge N, Woodrow J, Friel J, Hoff A, Gagel R, et al. Ablation of calcitonin/calcitonin gene-related peptide-alpha impairs fetal magnesium but not calcium homeostasis. Am J Physiol Endocrinol Metab. 2004;287:E218-26 pubmed
    ..These findings indicate that calcitonin and CGRPalpha are not needed for normal fetal calcium metabolism but may regulate aspects of fetal Mg metabolism. ..
  31. Klatt A, Paulsson M, Wagener R. Expression of matrilins during maturation of mouse skeletal tissues. Matrix Biol. 2002;21:289-96 pubmed
    ..During joint development matrilin-2 and matrilin-4 are present at the developing joint surface, while in articular cartilage of 6-week-old mice all matrilins are only weakly expressed. ..