| Antigen/Gene or Protein Targets | HD gene; Tet-On GFP-HD (23 Gln repeats) |
| Parental Line | PC-12 |
| Host | Rat |
| Disease Keywords | Huntington’s disease; neurodegeneration; neuroendocrine tumour |
| Relevance | The PC-12 HD-Q23 cell lines 14 and 7 can be used for in-vitro assay development for neurodegenerative diseases. The huntingtin gene is variable in its structure, as the many polymorphisms of the gene can lead to variable numbers of glutamine residues present in the protein. In its wild-type (normal) form, it contains 6-35 glutamine residues. However, in individuals affected by Huntington's disease (an autosomal dominant genetic disorder), it contains more than 36 glutamine residues (highest reported repeat length is about 250). The exact function of this protein is not known, but it plays an important role in nerve cells homeostasis. |
| Production Details | Inducible lines in PC12 (rat phaeochromocytoma) cells, which express a GFP-tagged exon 1 fragment of the HD gene with 23 glutamine repeats (cell lines 14 and 7), driven by a doxycycline (dox)-dependent Tet-On promoter. |
| Research Area | Neurobiology |
| Recommended Growing Conditions | Cells maintained at 75 µg/ml hygromycin in standard medium consisting of high glucose DMEM with 100 U/ml penicillin/streptomycin, 2 mM L-glutamine, 10% heat-inactivated horse serum (HS) , 5% Tet-approved fetal bovine serum (FBS) and 100 µg/ml G418 at 37°C, 10% CO2. |
| Cellosaurus ID | CVCL_AU97 CVCL_AU98 |
Invented by David Rubensztein at University of Cambridge
|
David Rubensztein |
