Couillard-Despres, Sebastien and Uyanik, Goekhan and Ploetz, Sonja and Karl, Claudia and Koch, Hartmut and Winkler, Juergen and Aigner, Ludwig (2004) Mitotic impairment by doublecortin is diminished by doublecortin mutations found in patients. NEUROGENETICS, 5 (2). pp. 83-93. ISSN 1364-6745, 1364-6753
Full text not available from this repository. (Request a copy)Abstract
Mutations in doublecortin (DCX) affect the migration of neuronal precursor cells and cause subcortical band heterotopia and lissencephaly. DCX is known to bind and bundle microtubules; however, the impact of mutation on DCX function and its relation to the manifestation of DCX-associated disorders is still unclear. We analyzed the impact of DCX mutants on COS7 cell microtubule networks. We found that both mutant and wild type DCX are able to bind and bundle microtubules; however, mutants possess a decreased ability to perturb the mitotic machinery, to cause abnormal spindle orientation, and to impair mitotic progression. The magnitude of this decrease is proportional to the severity of the mutation-associated clinical symptoms, thereby providing a cell-based assay for the prognosis of DCX-associated neuronal migration disorders.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | SUBCORTICAL BAND HETEROTOPIA; DOUBLE-CORTEX SYNDROME; MICROTUBULE-ASSOCIATED PROTEIN; SOMATIC MOSAICISM; FUNCTIONAL DOMAINS; DCX GENE; LIS1; MAP2; TAU; MALFORMATION; neuronal migration; neurogenesis; neuronal precursor cells; cell division; double cortex syndrome |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 20 Jul 2021 04:42 |
| Last Modified: | 20 Jul 2021 04:42 |
| URI: | https://pred.uni-regensburg.de/id/eprint/37608 |
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