PROSITE documentation PDOC50309
Doublecortin domain profile


X-linked lissencephaly is a severe brain malformation affecting males. Recently it has been demonstrated that the doublecortin gene is implicated in this disorder [1]. Doublecortin was found to bind to the microtubule cytoskeleton. In vivo and in vitro assays show that Doublecortin stabilizes microtubules and causes bundling [2]. Doublecortin is a basic protein with an iso-electric point of 10, typical of microtubule-binding proteins. However, its sequence contains no known microtubule-binding domain(s).

The detailed sequence analysis of Doublecortin and Doublecortin-like proteins allowed the identification of an evolutionarily conserved Doublecortin (DC) domain. This domain is found in the N-terminus of proteins and consists of one or two tandemly repeated copies of an around 80 amino acids region [3]. It has been suggested that the first DC domain of Doublecortin binds tubulin and enhances microtubule polymerization [3].

Some proteins known to contain a DC domain are listed below:

  • Doublecortin. It is required for neuronal migration [1]. A large number of point mutations in the human DCX gene leading to lissencephaly are located within the DC domains [3].
  • Human serine/threonine-protein kinase DCAMKL1. It is a probable kinase that may be involved in a calcium-signaling pathway controling neuronal migration in the developing brain [4].
  • Retinitis pigmentosa 1 protein. It could play a role in the differentiation of photoreceptor cells. Mutation in the human RP1 gene cause retinitis pigmentosa of type 1 [5].
Last update:

December 2001 / First entry.


Technical section

PROSITE method (with tools and information) covered by this documentation:

DC, PS50309; Doublecortin domain profile  (MATRIX)


1AuthorsDes Portes V. Pinard J.M. Billuart P. Vinet M.C. Koulakoff A. Carrie A. Gelot A. Dupuis E. Motte J. Berwald-Netter Y. Catala M. Kahn A. Beldjord C. Chelly J.
SourceCell 92:51-61(1998).

2AuthorsHoresh D. Sapir T. Francis F. Wolf S.G. Caspi M. Elbaum M. Chelly J. Reiner O.
TitleDoublecortin, a stabilizer of microtubules.
SourceHum. Mol. Genet. 8:1599-1610(1999).
PubMed ID10441322

3AuthorsSapir T. Horesh D. Caspi M. Atlas R. Burgess H.A. Wolf S.G. Francis F. Chelly J. Elbaum M. Pietrokovski S. Reiner O.
TitleDoublecortin mutations cluster in evolutionarily conserved functional domains.
SourceHum. Mol. Genet. 9:703-712(2000).
PubMed ID10749977

4AuthorsBurgess H.A. Martinez S. Reiner O.
TitleKIAA0369, doublecortin-like kinase, is expressed during brain development.
SourceJ. Neurosci. Res. 58:567-575(1999).
PubMed ID10533048

5AuthorsGuillonneau X. Piriev N.I. Danciger M. Kozak C.A. Cideciyan A.V. Jacobson S.G. Farber D.B.
TitleA nonsense mutation in a novel gene is associated with retinitis pigmentosa in a family linked to the RP1 locus.
SourceHum. Mol. Genet. 8:1541-1546(1999).
PubMed ID10401003

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