Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel.

doi:10.1371/journal.pone.0032654

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Article

Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel.

PloS one - 1 Jan 2012

Harley Carol A, Jesus Catarina S H, Carvalho Ricardo, Brito Rui M M, Morais-Cabral João H

Abstract excerpt

Inherited human long-QT2 syndrome (LQTS) results from mutations in the gene encoding the HERG channel. Several LQT2-associated mutations have been mapped to the amino terminal cytoplasmic Per-Arnt-Sim (PAS) domain of the HERG1a channel subunit. Here we have characterized the trafficking properties of some LQT2-associated PAS domain mutants and analyzed rescue of the trafficking mutants by low temperature (27°C)...

Topics

Circular Dichroism
ERG1 Potassium Channel
Ether-A-Go-Go Potassium Channels
Glycoside Hydrolases
HEK293 Cells
Humans
Long QT Syndrome
Mutation
Phenotype
Protein Denaturation
Protein Folding

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Protein Structure, Secondary

Protein Structure, Tertiary

Protein Transport

Temperature

Thermodynamics

Ultraviolet Rays

Source

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Identifiers

PMID: 22396785
DOI: 10.1371/journal.pone.0032654
PMCID: PMC3292575

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Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel.PMID 22396785Read ↗

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