Modulation of Kv4.2/KChIP3 interaction by the ceroid lipofuscinosis neuronal 3 protein CLN3.
Amino Acid Substitution
Gene Expression Regulation
/ genetics
HEK293 Cells
Humans
Kv Channel-Interacting Proteins
/ genetics
Membrane Glycoproteins
Molecular Chaperones
Mutation, Missense
Neuronal Ceroid-Lipofuscinoses
/ genetics
Protein Binding
Repressor Proteins
/ genetics
Shal Potassium Channels
/ biosynthesis
Batten disease
KChIP
Kv channel-interacting protein
biophysics
calcium
gating
juvenile neuronal ceroid lipofuscinosis (JNCL)
neurodegenerative disease
patch–clamp
potassium channel
protein complex
Journal
The Journal of biological chemistry
ISSN: 1083-351X
Titre abrégé: J Biol Chem
Pays: United States
ID NLM: 2985121R
Informations de publication
Date de publication:
21 08 2020
21 08 2020
Historique:
received:
10
04
2020
revised:
22
06
2020
pubmed:
10
7
2020
medline:
20
1
2021
entrez:
10
7
2020
Statut:
ppublish
Résumé
Voltage-gated potassium (Kv) channels of the Kv4 subfamily associate with Kv channel-interacting proteins (KChIPs), which leads to enhanced surface expression and shapes the inactivation gating of these channels. KChIP3 has been reported to also interact with the late endosomal/lysosomal membrane glycoprotein CLN3 (ceroid lipofuscinosis neuronal 3), which is modified because of gene mutation in juvenile neuronal ceroid lipofuscinosis (JNCL). The present study was undertaken to find out whether and how CLN3, by its interaction with KChIP3, may indirectly modulate Kv4.2 channel expression and function. To this end, we expressed KChIP3 and CLN3, either individually or simultaneously, together with Kv4.2 in HEK 293 cells. We performed co-immunoprecipitation experiments and found a lower amount of KChIP3 bound to Kv4.2 in the presence of CLN3. In whole-cell patch-clamp experiments, we examined the effects of CLN3 co-expression on the KChIP3-mediated modulation of Kv4.2 channels. Simultaneous co-expression of CLN3 and KChIP3 with Kv4.2 resulted in a suppression of the typical KChIP3-mediated modulation;
Identifiants
pubmed: 32641494
pii: S0021-9258(17)50071-X
doi: 10.1074/jbc.RA120.013828
pmc: PMC7443505
pii:
doi:
Substances chimiques
CLN3 protein, human
0
KCNIP3 protein, human
0
Kv Channel-Interacting Proteins
0
Membrane Glycoproteins
0
Molecular Chaperones
0
Repressor Proteins
0
Shal Potassium Channels
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
12099-12110Informations de copyright
© 2020 Seifert et al.
Déclaration de conflit d'intérêts
Conflict of interest—The authors declare that they have no conflicts of interest with the contents of this article.
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