Cells exposed to a huntingtin fragment containing an expanded polyglutamine tract show no sign of ion channel formation: results arguing against the ion channel hypothesis.

TY - JOUR

T1 - Cells exposed to a huntingtin fragment containing an expanded polyglutamine tract show no sign of ion channel formation: results arguing against the ion channel hypothesis.

AU - Nørremølle, Anne

AU - Grunnet, Morten

AU - Hasholt, Lis

AU - Sørensen, Sven Asger

N1 - Keywords: Animals; Blotting, Western; CHO Cells; Cricetinae; Green Fluorescent Proteins; Humans; Huntington Disease; Ion Channels; Luminescent Proteins; Membrane Potentials; Nerve Tissue Proteins; Nuclear Proteins; Oocytes; Patch-Clamp Techniques; Peptides; Recombinant Proteins; Time Factors; Transfection; Trinucleotide Repeat Expansion; Xenopus

PY - 2003

Y1 - 2003

N2 - Ion channels formed by expanded polyglutamine tracts have been proposed to play an important role in the pathological processes leading to neurodegeneration in Huntington's disease and other CAG repeat diseases. We tested the capacity of a huntingtin fragment containing an expanded polyglutamine tract to form ion channels in two cell types. Whole cell current from Xenopus oocytes was recorded using two-electrode voltage-clamp technique, and whole cell current from CHO-K1 cells was recorded by patch-clamp technique. The fragment with an expanded polyglutamine sequence induced no change in the currents recorded in any of the two expression systems, indicating no changes in ion channel activity. The results therefore argue against the proposed hypothesis of expanded polyglutamines forming ion channels.

AB - Ion channels formed by expanded polyglutamine tracts have been proposed to play an important role in the pathological processes leading to neurodegeneration in Huntington's disease and other CAG repeat diseases. We tested the capacity of a huntingtin fragment containing an expanded polyglutamine tract to form ion channels in two cell types. Whole cell current from Xenopus oocytes was recorded using two-electrode voltage-clamp technique, and whole cell current from CHO-K1 cells was recorded by patch-clamp technique. The fragment with an expanded polyglutamine sequence induced no change in the currents recorded in any of the two expression systems, indicating no changes in ion channel activity. The results therefore argue against the proposed hypothesis of expanded polyglutamines forming ion channels.

U2 - 10.1002/jnr.10468

DO - 10.1002/jnr.10468

M3 - Journal article

C2 - 12478622

SN - 0360-4012

VL - 71

SP - 132

EP - 137

JO - Journal of Neuroscience Research

JF - Journal of Neuroscience Research

IS - 1

ER -