Fragile X mental retardation protein controls gating of the sodium-activated potassium channel Slack.
In humans, the absence of Fragile X mental retardation protein (FMRP), an RNA-binding protein, results in Fragile X syndrome, the most common inherited form of intellectual disability. Using biochemical and electrophysiological studies, we found that FMRP binds to the C terminus of the Slack sodium-activated potassium channel to activate the channel in mice. Our findings suggest that Slack activity provides a link between patterns of neuronal firing and changes in protein translation.
Department of Pharmacology, Yale University School of Medicine, New Haven, Connecticut, USA.
Maile R
MR
Brown
Jack
J
Kronengold
Valeswara-Rao
VR
Gazula
Yi
Y
Chen
John G
JG
Strumbos
Fred J
FJ
Sigworth
Dhasakumar
D
Navaratnam
Leonard K
LK
Kaczmarek
D000818:Animals; D004195:Disease Models, Animal; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000502:physiology*; D015640:Ion Channel Gating / Q000502:physiology*; D051379:Mice; D009419:Nerve Tissue Proteins; D015221:Potassium Channels / Q000378:metabolism*; D000081033:Potassium Channels, Sodium-Activated
D051860:Fragile X Messenger Ribonucleoprotein 1; D009419:Nerve Tissue Proteins; D015221:Potassium Channels; D000081033:Potassium Channels, Sodium-Activated; C479624:Slo2 protein, mouse