collagen type xiii


Summary: A non-fibrillar collagen found as a ubiquitously expressed membrane- associated protein. Type XIII collagen contains both collagenous and non-collagenous domains along with a transmembrane domain within its N-terminal region.

Top Publications

  1. Logan C, Cossins J, Rodríguez Cruz P, Parry D, Maxwell S, Martínez Martínez P, et al. Congenital Myasthenic Syndrome Type 19 Is Caused by Mutations in COL13A1, Encoding the Atypical Non-fibrillar Collagen Type XIII α1 Chain. Am J Hum Genet. 2015;97:878-85 pubmed publisher
  2. Miyake M, Hori S, Morizawa Y, Tatsumi Y, Toritsuka M, Ohnishi S, et al. Collagen type IV alpha 1 (COL4A1) and collagen type XIII alpha 1 (COL13A1) produced in cancer cells promote tumor budding at the invasion front in human urothelial carcinoma of the bladder. Oncotarget. 2017;8:36099-36114 pubmed publisher
    ..Blocking of these collagens may be an attractive therapeutic approach for treatment of human urothelial cancer of the bladder. ..
  3. Maertens B, Hopkins D, Franzke C, Keene D, Bruckner Tuderman L, Greenspan D, et al. Cleavage and oligomerization of gliomedin, a transmembrane collagen required for node of ranvier formation. J Biol Chem. 2007;282:10647-59 pubmed
    ..Interestingly, the later cleavage induces formation of higher order, insoluble molecular aggregates that may play important roles in Na(+)-channel clustering. ..
  4. Gopinath B, Ma G, Wall J. Eye signs and serum eye muscle and collagen XIII antibodies in patients with transient and progressive thyroiditis. Thyroid. 2007;17:1123-9 pubmed
    ..There have been reports of the development of ophthalmopathy in patients with subacute thyroiditis (SAT) in the absence of Graves' disease and thyroid-stimulating hormone receptor (TSH-r) antibodies...
  5. Wall J, Lahooti H. [Pathogenesis of thyroid eye disease - does autoimmunity against the TSH receptor explain all cases?]. Endokrynol Pol. 2011;62 Suppl 1:1-7 pubmed
  6. Tjiang H, Lahooti H, McCorquodale T, Parmar K, Wall J. Eye and eyelid abnormalities are common in patients with Hashimoto's thyroiditis. Thyroid. 2010;20:287-90 pubmed publisher
    ..Although eyelid abnormalities may be a minor problem for most patients, for some there are major cosmetic implications requiring surgical management. ..
  7. Tahkola J, Rasanen J, Sund M, Mäkikallio K, Autio Harmainen H, Pihlajaniemi T. Cardiac dysfunction in transgenic mouse fetuses overexpressing shortened type XIII collagen. Cell Tissue Res. 2008;333:61-9 pubmed publisher
    ..Thus, overexpression of mutant type XIII collagen results in mid-gestation cardiac dysfunction in mouse fetuses, and these disturbances in cardiac function may lead to death in utero. ..
  8. Byun E, Caillier S, Montalban X, Villoslada P, Fernandez O, Brassat D, et al. Genome-wide pharmacogenomic analysis of the response to interferon beta therapy in multiple sclerosis. Arch Neurol. 2008;65:337-44 pubmed publisher
    ..In addition to new insights into the mechanistic biology of interferon beta, these results help define the molecular basis of interferon beta therapy response heterogeneity. ..
  9. Määttä M, Väisänen T, Väisänen M, Pihlajaniemi T, Tervo T. Altered expression of type XIII collagen in keratoconus and scarred human cornea: Increased expression in scarred cornea is associated with myofibroblast transformation. Cornea. 2006;25:448-53 pubmed
    ..Additionally, highly increased expression in scarred corneas suggests that it participates in the corneal wound healing process. ..

More Information


  1. Ylönen R, Kyrönlahti T, Sund M, Ilves M, Lehenkari P, Tuukkanen J, et al. Type XIII collagen strongly affects bone formation in transgenic mice. J Bone Miner Res. 2005;20:1381-93 pubmed
    ..The findings suggest that type XIII collagen has an important role in bone modeling, and in particular, it may have a function in coupling the regulation of bone mass to mechanical use. ..
  2. Väisänen M, Väisänen T, Pihlajaniemi T. The shed ectodomain of type XIII collagen affects cell behaviour in a matrix-dependent manner. Biochem J. 2004;380:685-93 pubmed
    ..The dual existence of membrane-bound and soluble variants widens our biological understanding of type XIII collagen. ..
  3. Väisänen T, Väisänen M, Pihlajaniemi T. Modulation of the cellular cholesterol level affects shedding of the type XIII collagen ectodomain. J Biol Chem. 2006;281:33352-62 pubmed
    ..This supports the hypothesis of type XIII collagen ectodomain also being cleaved intracellularly in the Golgi and suggests that the intracellular cleavage may act as a gating event in the vesicle-mediated ectodomain secretion. ..
  4. Snellman A, Tuomisto A, Koski A, Latvanlehto A, Pihlajaniemi T. The role of disulfide bonds and alpha-helical coiled-coils in the biosynthesis of type XIII collagen and other collagenous transmembrane proteins. J Biol Chem. 2007;282:14898-905 pubmed
    ..Moreover, furin cleavage at the NC1 domain can be expected in most of the proteins. ..
  5. Morris O, Schebitz Walter K, Telemo E, Hintschich C. Constitutively low expression of collagen XIII alpha 1 may help explain the vulnerability of the inferior rectus muscle to thyroid-associated ophthalmopathy. Orbit. 2016;35:343-349 pubmed
  6. Morris O, Schebitz Walter K, Telemo E, Hintschich C. Overexpression of collagen XIII in extraocular fat affected by active thyroid-associated ophthalmopathy: A crucial piece of the puzzle?. Orbit. 2016;35:227-32 pubmed publisher
    ..We identified overexpression of collagen XIII in active TAO-affected fat. We discuss how species and cell-type specific responses of collagen XIII to stressors may help explain the different phenotypes of TAO. ..
  7. Väisänen M, Väisänen T, Tu H, Pirilä P, Sormunen R, Pihlajaniemi T. The shed ectodomain of type XIII collagen associates with the fibrillar fibronectin matrix and may interfere with its assembly in vitro. Biochem J. 2006;393:43-50 pubmed
    ..The results presented here imply that the shedding of the type XIII collagen ectodomain results in a biologically active molecule capable of remodelling the structure of the pericellular matrix. ..
  8. De Bellis A, Sansone D, Coronella C, Conte M, Iorio S, Perrino S, et al. Serum antibodies to collagen XIII: a further good marker of active Graves' ophthalmopathy. Clin Endocrinol (Oxf). 2005;62:24-9 pubmed
    ..Thus, the evaluation of these antibodies could be added to other known markers as a useful and inexpensive tool in monitoring Graves' patients and in modulating the treatment of GO. ..
  9. Latvanlehto A, Fox M, Sormunen R, Tu H, Oikarainen T, Koski A, et al. Muscle-derived collagen XIII regulates maturation of the skeletal neuromuscular junction. J Neurosci. 2010;30:12230-41 pubmed publisher
    ..Altogether, the results presented here reveal that collagen XIII is a novel muscle-derived cue necessary for the maturation and function of the vertebrate NMJ. ..
  10. Tu H, Sasaki T, Snellman A, Göhring W, Pirilä P, Timpl R, et al. The type XIII collagen ectodomain is a 150-nm rod and capable of binding to fibronectin, nidogen-2, perlecan, and heparin. J Biol Chem. 2002;277:23092-9 pubmed
  11. Latvanlehto A, Snellman A, Tu H, Pihlajaniemi T. Type XIII collagen and some other transmembrane collagens contain two separate coiled-coil motifs, which may function as independent oligomerization domains. J Biol Chem. 2003;278:37590-9 pubmed
    ..All in all, we propose that two widely separated coiled-coil domains of type XIII and related collagens function as independent oligomerization domains participating in the folding of distinct areas of the molecule. ..
  12. Dennis J, Meehan D, Delimont D, Zallocchi M, Perry G, O Brien S, et al. Collagen XIII induced in vascular endothelium mediates alpha1beta1 integrin-dependent transmigration of monocytes in renal fibrosis. Am J Pathol. 2010;177:2527-40 pubmed publisher
  13. Harvey S, Miner J. Revisiting the glomerular charge barrier in the molecular era. Curr Opin Nephrol Hypertens. 2008;17:393-8 pubmed publisher
    ..In contrast, cell-associated sialoproteins are clearly important, but whether their functions actually involve contributions to the charge barrier is unknown. ..
  14. Kvist A, Latvanlehto A, Sund M, Eklund L, Väisänen T, Hagg P, et al. Lack of cytosolic and transmembrane domains of type XIII collagen results in progressive myopathy. Am J Pathol. 2001;159:1581-92 pubmed
    ..Thus type XIII collagen seems to participate in the linkage between muscle fiber and basement membrane, a function impaired by lack of the cytosolic and transmembrane domains. ..
  15. Gopinath B, Adams C, Musselman R, Tani J, Wall J. Antibodies against calsequestrin and type XIII collagen are good markers for chronic upper eyelid retraction. Ocul Immunol Inflamm. 2007;15:81-8 pubmed
    ..The mix of antibodies against calsequestrin and collagen XIII may shed light on the diverse presentations found in thyroid-associated ophthalmopathy. ..
  16. Tuomisto A, Sund M, Tahkola J, Latvanlehto A, Savolainen E, Autio Harmainen H, et al. A mutant collagen XIII alters intestinal expression of immune response genes and predisposes transgenic mice to develop B-cell lymphomas. Cancer Res. 2008;68:10324-32 pubmed publisher
    ..These results define collagen XIII-dependent maintenance of the intestinal BM as a previously unappreciated component of immune responses and a critical determinant of cancer susceptibility. ..
  17. Heikkinen A, Tu H, Pihlajaniemi T. Collagen XIII: a type II transmembrane protein with relevance to musculoskeletal tissues, microvessels and inflammation. Int J Biochem Cell Biol. 2012;44:714-7 pubmed publisher
    ..Recent in vivo studies highlight its involvement in the development, differentiation and maturation of musculoskeletal tissues and vessels and in maintaining tissue integrity. ..
  18. Väisänen T, Väisänen M, Autio Harmainen H, Pihlajaniemi T. Type XIII collagen expression is induced during malignant transformation in various epithelial and mesenchymal tumours. J Pathol. 2005;207:324-35 pubmed