Abstract:
:The potassium channel tetramerization domain-containing protein 7 (KCTD7) was named after the structural homology of its predicted N-terminal broad complex, tramtrack and bric à brac/poxvirus and zinc finger domain with the T1 domain of the Kv potassium channel, but its expression profile and cellular function are still largely unknown. We have recently reported a homozygous nonsense mutation of KCTD7 in patients with a novel form of autosomal recessive progressive myoclonic epilepsy. Here, we show that KCTD7 expression hyperpolarizes the cell membrane and reduces the excitability of transfected neurons in patch clamp experiments. We found the expression of KCTD7 in the hippocampal and Purkinje cells of the murine brain, an expression profile consistent with our patients' phenotype. The effect on the plasma membrane resting potential is possibly mediated by Cullin-3, as we demonstrated direct molecular interaction of KCTD7 with Cullin-3 in co-immunoprecipitation assays. Our data link progressive myoclonic epilepsy to an inherited defect of the neuron plasma membrane's resting potential in the brain.
journal_name
Mol Neurobioljournal_title
Molecular neurobiologyauthors
Azizieh R,Orduz D,Van Bogaert P,Bouschet T,Rodriguez W,Schiffmann SN,Pirson I,Abramowicz MJdoi
10.1007/s12035-011-8194-0subject
Has Abstractpub_date
2011-08-01 00:00:00pages
111-21issue
1eissn
0893-7648issn
1559-1182journal_volume
44pub_type
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