Sophion

KCNQ4
Voltage dependent: KQT related

(Alternative nomenclature: Kv7.4)

KCNQ4 is a slow delayed rectifier voltage-gated 6-TM 1-P type potassium channel. Homotetrameric.

Localization:
Cochlea: Sensory outer, but not inner hair cells; vestibular organs, brainstem: auditory nuclei

Information links:
http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=603537

QPatch recording of KCNQ4 currents:
(Courtesy of Dr. Mads P.G. Korsgaard, NeuroSearch)


Activation of KCNQ4 whole-cell currents by depolarizations from -90 to +40 mV from a holding potential of -100 mV. Expression system: HEK-293.


I-V relationship based on the KCNQ4 whole-cell currents presented above.

Pharmacological profiling:
Inhibitors: Linopirdine, DMP543, XE-991
Activators: Retigabine, BMS-204352
(The list is not intended to be complete)

QPatch concentration-response analysis:
(Courtesy of Dr. Mads P.G. Korsgaard, NeuroSearch)


Effect of on the KCNQ activator retigabine (30 μM) on KCNQ4 whole-cell currents (red traces)


I-V relationschips with (red) or without retigabine based on currents shown above.

Pathophysiology:  
Nonsyndromic sensorineural hearing loss, dominant (DFNA2)

Recent review articles:
Gribkoff VK. The therapeutic potential of neuronal KCNQ channel modulators. Expert Opin Ther Targets;7:737-48, 2003.

QPatch written material:
N/A

 

 


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