PMID- 11223304
OWN - NLM
STAT- MEDLINE
DCOM- 20010628
LR  - 20190822
IS  - 0378-5955 (Print)
IS  - 0378-5955 (Linking)
VI  - 153
IP  - 1-2
DP  - 2001 Mar
TI  - KCNQ1/KCNE1 potassium channels in mammalian vestibular dark cells.
PG  - 132-45
AB  - The high [K(+)] in the inner ear endolymph is essential for mechanosensory 
      transduction in hearing and balance. Several ion channels, including a slowly 
      activating, voltage-dependent, outwardly conducting K(+) channel composed of the 
      KCNQ1 (KvLQT1) and KCNE1 (IsK/minK) subunits, are expressed at the apical surface 
      of vestibular dark cells. We investigated the underlying molecular mechanisms of 
      this conductance using in situ hybridization, RT-PCR, and immunocytochemistry and 
      by tracking the ultrastructural changes of vestibular structures in kcne1(-/-) 
      mice. In the wild type mice, the KCNE1 and KCNQ1 proteins are expressed 
      specifically at the apical membrane of dark cells, as early as gestational day 
      (GD) 17 for KCNE1 while KCNQ1 mRNAs can be detected at GD 18. This is the first 
      demonstration that the two protein components of this potassium channel 
      co-localize in a polarized fashion at the cellular level. Although the vestibular 
      end-organs are normal at birth in kcne1(-/-) mice, they begin to show 
      modifications during postnatal development: we observed an increase in the height 
      of the dark cells, in their number of mitochondria, and in basolateral membrane 
      infoldings. Subsequently, the epithelium degenerates and the endolymphatic space 
      collapses. Similar changes are known to occur in the cardio-auditory 
      Jervell--Lange-Nielsen syndrome which is caused by mutations in the same channel.
FAU - Nicolas, M
AU  - Nicolas M
AD  - INSERM U432, Universite Montpellier 2, France.
FAU - Dememes, D
AU  - Dememes D
FAU - Martin, A
AU  - Martin A
FAU - Kupershmidt, S
AU  - Kupershmidt S
FAU - Barhanin, J
AU  - Barhanin J
LA  - eng
PT  - Journal Article
PL  - Netherlands
TA  - Hear Res
JT  - Hearing research
JID - 7900445
RN  - 0 (DNA Primers)
RN  - 0 (KCNQ Potassium Channels)
RN  - 0 (KCNQ1 Potassium Channel)
RN  - 0 (KCNQ1 protein, human)
RN  - 0 (Kcnq1 protein, mouse)
RN  - 0 (Kcnq1 protein, rat)
RN  - 0 (Potassium Channels)
RN  - 0 (Potassium Channels, Voltage-Gated)
RN  - 0 (RNA, Messenger)
RN  - 0 (potassium channel protein I(sk))
SB  - IM
MH  - Amino Acid Sequence
MH  - Animals
MH  - Base Sequence
MH  - DNA Primers/genetics
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization
MH  - KCNQ Potassium Channels
MH  - KCNQ1 Potassium Channel
MH  - Long QT Syndrome/genetics/metabolism/pathology
MH  - Mice
MH  - Mice, Knockout
MH  - Microscopy, Electron
MH  - Molecular Sequence Data
MH  - Mutation
MH  - Potassium Channels/genetics/*metabolism
MH  - *Potassium Channels, Voltage-Gated
MH  - RNA, Messenger/genetics/metabolism
MH  - Rats
MH  - Rats, Sprague-Dawley
MH  - Reverse Transcriptase Polymerase Chain Reaction
MH  - Vestibule, Labyrinth/cytology/growth & development/*metabolism
EDAT- 2001/02/27 10:00
MHDA- 2001/06/29 10:01
CRDT- 2001/02/27 10:00
PHST- 2001/02/27 10:00 [pubmed]
PHST- 2001/06/29 10:01 [medline]
PHST- 2001/02/27 10:00 [entrez]
AID - S0378595500002689 [pii]
AID - 10.1016/s0378-5955(00)00268-9 [doi]
PST - ppublish
SO  - Hear Res. 2001 Mar;153(1-2):132-45. doi: 10.1016/s0378-5955(00)00268-9.