When cilia go bad: cilia defects and ciliopathies - PubMed (original) (raw)

Review

doi: 10.1038/nrm2278.

Affiliations

• PMID: 17955020
• DOI: 10.1038/nrm2278

Review

When cilia go bad: cilia defects and ciliopathies

Manfred Fliegauf et al. Nat Rev Mol Cell Biol. 2007 Nov.

Erratum in

• Nat Rev Mol Cell Biol. 2008 Jan;9(1):88

Abstract

Defects in the function of cellular organelles such as peroxisomes, lysosomes and mitochondria are well-known causes of human diseases. Recently, another organelle has also been added to this list. Cilia--tiny hair-like organelles attached to the cell surface--are located on almost all polarized cell types of the human body and have been adapted as versatile tools for various cellular functions, explaining why cilia-related disorders can affect many organ systems. Several molecular mechanisms involved in cilia-related disorders have been identified that affect the structure and function of distinct cilia types.

PubMed Disclaimer

Similar articles

• Ciliary biology: understanding the cellular and genetic basis of human ciliopathies.
  Cardenas-Rodriguez M, Badano JL. Cardenas-Rodriguez M, et al. Am J Med Genet C Semin Med Genet. 2009 Nov 15;151C(4):263-80. doi: 10.1002/ajmg.c.30227. Am J Med Genet C Semin Med Genet. 2009. PMID: 19876935 Review.
• Novel tools to unravel molecular mechanisms in cilia-related disorders.
  Fliegauf M, Omran H. Fliegauf M, et al. Trends Genet. 2006 May;22(5):241-5. doi: 10.1016/j.tig.2006.03.002. Epub 2006 Mar 24. Trends Genet. 2006. PMID: 16564109 Review.
• The roles of cilia in developmental disorders and disease.
  Bisgrove BW, Yost HJ. Bisgrove BW, et al. Development. 2006 Nov;133(21):4131-43. doi: 10.1242/dev.02595. Epub 2006 Oct 4. Development. 2006. PMID: 17021045 Review.
• [The concept of "organellopathies"--component of modern cellular pathology].
  David H. David H. Zentralbl Allg Pathol. 1990;136(1-2):15-32. Zentralbl Allg Pathol. 1990. PMID: 2183527 Review. German.
• Ciliary syndromes and treatment.
  Klysik M. Klysik M. Pathol Res Pract. 2008;204(2):77-88. doi: 10.1016/j.prp.2007.10.013. Pathol Res Pract. 2008. PMID: 18312782 Review.

Cited by

• Ulk4 Is Essential for Ciliogenesis and CSF Flow.
  Liu M, Guan Z, Shen Q, Lalor P, Fitzgerald U, O'Brien T, Dockery P, Shen S. Liu M, et al. J Neurosci. 2016 Jul 20;36(29):7589-600. doi: 10.1523/JNEUROSCI.0621-16.2016. J Neurosci. 2016. PMID: 27445138 Free PMC article.
• Enteric neurons show a primary cilium.
  Luesma MJ, Cantarero I, Castiella T, Soriano M, Garcia-Verdugo JM, Junquera C. Luesma MJ, et al. J Cell Mol Med. 2013 Jan;17(1):147-53. doi: 10.1111/j.1582-4934.2012.01657.x. Epub 2012 Dec 4. J Cell Mol Med. 2013. PMID: 23205631 Free PMC article.
• ADAMTS9-Mediated Extracellular Matrix Dynamics Regulates Umbilical Cord Vascular Smooth Muscle Differentiation and Rotation.
  Nandadasa S, Nelson CM, Apte SS. Nandadasa S, et al. Cell Rep. 2015 Jun 16;11(10):1519-28. doi: 10.1016/j.celrep.2015.05.005. Epub 2015 May 28. Cell Rep. 2015. PMID: 26027930 Free PMC article.
• The molecular structure of mammalian primary cilia revealed by cryo-electron tomography.
  Kiesel P, Alvarez Viar G, Tsoy N, Maraspini R, Gorilak P, Varga V, Honigmann A, Pigino G. Kiesel P, et al. Nat Struct Mol Biol. 2020 Dec;27(12):1115-1124. doi: 10.1038/s41594-020-0507-4. Epub 2020 Sep 28. Nat Struct Mol Biol. 2020. PMID: 32989303 Free PMC article.
• Molecular and cellular basis of left-right asymmetry in vertebrates.
  Hamada H. Hamada H. Proc Jpn Acad Ser B Phys Biol Sci. 2020;96(7):273-296. doi: 10.2183/pjab.96.021. Proc Jpn Acad Ser B Phys Biol Sci. 2020. PMID: 32788551 Free PMC article. Review.

Publication types

MeSH terms

LinkOut - more resources

• Full Text Sources

  • Nature Publishing Group

• Other Literature Sources

  • The Lens - Patent Citations Database

• Research Materials

  • NCI CPTC Antibody Characterization Program