Doublecortin (Dcx) Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
Overview
This page provides comprehensive information about this protein. See the content below for detailed information.
Doublecortin (Dcx) Protein is an important component in the neurobiology of neurodegenerative diseases. This page provides detailed information about its structure, function, and role in disease processes.
Overview
This page provides comprehensive information about this protein. See the content below for detailed information.
Doublecortin is a microtubule-associated protein essential for neuronal migration and cortical lamination. Mutations cause lissencephaly and double [cortex](/brain-regions/cortex) syndrome, highlighting its critical role in brain development.
Severe developmental delay - profound intellectual disability
Seizures - early-onset epilepsy
Males - more severely affected
Double Cortex Syndrome
Subcortical band heterotopia in females
Milder phenotype than lissencephaly
X-linked dominant inheritance
Mosaic expression
Periventricular Heterotopia
DCX mutations in some cases
Nodular gray matter heterotopia
Seizures and developmental delay
Neurodegeneration
Altered DCX expression in AD
May affect neuronal connectivity
Potential regenerative role
Therapeutic Approaches
Gene therapy - restore DCX expression
Microtubule stabilizers - compensate for DCX loss
Seizure management - antiepileptic drugs
Supportive care - developmental support
Key Publications
des Portes V, et al. (1998). "A novel CNS gene required for neuronal migration and involved in X-linked subcortical laminar heterotopia and lissencephaly syndrome." Cell. 92(1):51-61. [DOI:10.1016/s0092-8674(00](https://doi.org/10.1016/s0092-8674(00))80898-3
Gleeson JG, et al. (1998). "Doublecortin, a brain-specific gene mutated in human X-linked lissencephaly and double cortex syndrome, encodes a putative signaling protein." Cell. 92(1):63-72. [DOI:10.1016/s0092-8674(00](https://doi.org/10.1016/s0092-8674(00))80899-5
Koizumi H, et al. (2006). "Doublecortin maintains bipolar shape and nuclear translocation during migration in the adult forebrain." Nat Neurosci. 9(6):779-787. [DOI:10.1038/nn1704](https://doi.org/10.1038/nn1704)
[PubMed - Research Papers](https://pubmed.ncbi.nlm.nih.gov/)
[Allen Brain Atlas](https://brain-map.org/)
[BrainSpan Atlas](https://brainspan.org/)
See Also
[Cell Types Index](/cell-types)cell-types)
[Brain Regions Index](/brain-regions)brain-regions)
Background
The study of Doublecortin (Dcx) Protein has evolved significantly over the past decades. Research in this area has revealed important insights into the underlying mechanisms of neurodegeneration and continues to drive therapeutic development.
Historical context and key discoveries in this field have shaped our current understanding and will continue to guide future research directions.
References
[Gleeson JG, Lin PT, Flanagan KA, Walsh CA, "Doublecortin is a microtubule-associated protein expressed in migrating and differentiating neurons." Neuron (1999)](https://doi.org/10.1016/S0896-6273(00)
[Francis F, Koulakoff A, Boucher D, Chafey P, Schaar B, et al, "Doublecortin is a developmentally regulated, microtubule-associated protein expressed in migrating and differentiating neurons." Neuron (1999)](https://doi.org/10.1016/S0896-6273(00)
[Koizumi H, Higginbotham H, Pemble M, Endo S M, Goldman, et al, "Doublecortin maintains neuronal polarity in vivo and in vitro." Nat Commun (2016)](https://doi.org/10.1038/ncomms11043)
[Stott SR, Barker RA, "Doublecortin-expressing cells are associated with neurogenesis in the adult brain." Cell Tissue Res (2015)](https://doi.org/10.1007/s00441-014-2063-x)
[Matsumoto N, Tanaka S, Ohshima Y, Yamada M, Hirohashi T, et al, "DCX and LIS1 in neuronal migration and development." Neurosci Res (2019)](https://doi.org/10.1016/j.neures.2018.12.005)