A Patient Suffering from Hypokalemic Periodic Paralysis Is Deficient in Skeletal Muscle ATP-sensitive K+ channels

Sofija Jovanovic; Qingyou Du; Somnath Mukhopadhyay; Robert Swingler; Richard Buckley; Jane McEachen; Aleksandar Jovanovic

doi:10.1111/j.1752-8062.2008.00007.x

A Patient Suffering from Hypokalemic Periodic Paralysis Is Deficient in Skeletal Muscle ATP-sensitive K+ channels

Sofija Jovanovic, Qingyou Du, Somnath Mukhopadhyay, Robert Swingler, Richard Buckley, Jane McEachen, Aleksandar Jovanovic

Research output: Contribution to journal › Article › peer-review

19 Citations (Scopus)

Abstract

Hypokalemic periodic paralysis (HOPP) is a rare disease associated with attacks of muscle weakness and hypokalemia. In the present study, immunoprecipitation/Western blotting has shown that a HOPP patient was deficient in sarcolemmal K-ATP channels. Real-time RT-PCR has revealed that HOPP has decreased mRNA levels of Kir6.2, a pore-forming K-ATP channel subunit, without affecting the expression of other K-ATP channel-forming proteins. Based on these findings, we conclude that HOPP could be associated with impaired expression of Kir6.2 which leads to deficiency in skeletal muscle K-ATP channels, which may explain the symptoms and clinical signs of this disease.

Original language	English
Pages (from-to)	71-74
Number of pages	4
Journal	Clinical and Translational Science
Volume	1
Issue number	1
DOIs	https://doi.org/10.1111/j.1752-8062.2008.00007.x
Publication status	Published - May 2008

Keywords

hypokalemic periodic paralysis
K-ATP channels
skeletal muscle
Kir6.2
POTASSIUM CHANNELS
H9C2 CELLS
EXPRESSION
ISCHEMIA
SUBUNIT
SERVES
KINASE

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10.1111/j.1752-8062.2008.00007.x

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title = "A Patient Suffering from Hypokalemic Periodic Paralysis Is Deficient in Skeletal Muscle ATP-sensitive K+ channels",

abstract = "Hypokalemic periodic paralysis (HOPP) is a rare disease associated with attacks of muscle weakness and hypokalemia. In the present study, immunoprecipitation/Western blotting has shown that a HOPP patient was deficient in sarcolemmal K-ATP channels. Real-time RT-PCR has revealed that HOPP has decreased mRNA levels of Kir6.2, a pore-forming K-ATP channel subunit, without affecting the expression of other K-ATP channel-forming proteins. Based on these findings, we conclude that HOPP could be associated with impaired expression of Kir6.2 which leads to deficiency in skeletal muscle K-ATP channels, which may explain the symptoms and clinical signs of this disease.",

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author = "Sofija Jovanovic and Qingyou Du and Somnath Mukhopadhyay and Robert Swingler and Richard Buckley and Jane McEachen and Aleksandar Jovanovic",

year = "2008",

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doi = "10.1111/j.1752-8062.2008.00007.x",

language = "English",

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T1 - A Patient Suffering from Hypokalemic Periodic Paralysis Is Deficient in Skeletal Muscle ATP-sensitive K+ channels

AU - Jovanovic, Sofija

AU - Du, Qingyou

AU - Mukhopadhyay, Somnath

AU - Swingler, Robert

AU - Buckley, Richard

AU - McEachen, Jane

AU - Jovanovic, Aleksandar

PY - 2008/5

Y1 - 2008/5

N2 - Hypokalemic periodic paralysis (HOPP) is a rare disease associated with attacks of muscle weakness and hypokalemia. In the present study, immunoprecipitation/Western blotting has shown that a HOPP patient was deficient in sarcolemmal K-ATP channels. Real-time RT-PCR has revealed that HOPP has decreased mRNA levels of Kir6.2, a pore-forming K-ATP channel subunit, without affecting the expression of other K-ATP channel-forming proteins. Based on these findings, we conclude that HOPP could be associated with impaired expression of Kir6.2 which leads to deficiency in skeletal muscle K-ATP channels, which may explain the symptoms and clinical signs of this disease.

AB - Hypokalemic periodic paralysis (HOPP) is a rare disease associated with attacks of muscle weakness and hypokalemia. In the present study, immunoprecipitation/Western blotting has shown that a HOPP patient was deficient in sarcolemmal K-ATP channels. Real-time RT-PCR has revealed that HOPP has decreased mRNA levels of Kir6.2, a pore-forming K-ATP channel subunit, without affecting the expression of other K-ATP channel-forming proteins. Based on these findings, we conclude that HOPP could be associated with impaired expression of Kir6.2 which leads to deficiency in skeletal muscle K-ATP channels, which may explain the symptoms and clinical signs of this disease.

KW - hypokalemic periodic paralysis

KW - K-ATP channels

KW - skeletal muscle

KW - Kir6.2

KW - POTASSIUM CHANNELS

KW - H9C2 CELLS

KW - EXPRESSION

KW - ISCHEMIA

KW - SUBUNIT

KW - SERVES

KW - KINASE

U2 - 10.1111/j.1752-8062.2008.00007.x

DO - 10.1111/j.1752-8062.2008.00007.x

M3 - Article

C2 - 20396605

SN - 1752-8054

VL - 1

SP - 71

EP - 74

JO - Clinical and Translational Science

JF - Clinical and Translational Science

IS - 1

ER -

A Patient Suffering from Hypokalemic Periodic Paralysis Is Deficient in Skeletal Muscle ATP-sensitive K+ channels

Abstract

Keywords

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