FKBP12.6 binding of ryanodine receptors carrying mutations associated with arrhythmogenic cardiac disease | Zendy

Spyros Zissimopoulos | Zendy; N. Lowri Thomas | Zendy; Wan W. Jamaluddin | Zendy; F. Anthony Lai | Zendy

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FKBP12.6 binding of ryanodine receptors carrying mutations associated with arrhythmogenic cardiac disease

Author(s) -

Spyros Zissimopoulos,

N. Lowri Thomas,

Wan W. Jamaluddin,

F. Anthony Lai

Publication year - 2009

Publication title -

biochemical journal

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.706

H-Index - 265

eISSN - 1470-8728

pISSN - 0264-6021

DOI - 10.1042/bj20082324

Subject(s) - ryanodine receptor , ryanodine receptor 2 , fkbp , biology , mutation , receptor , microbiology and biotechnology , medicine , endocrinology , genetics , gene

In the present paper we show that distinct human RyR2 (ryanodine receptor type 2) inherited mutations expressed in mammalian cells exhibit either unaltered or increased FKBP12.6 (12.6 kDa FK506-binding protein) binding compared with the wild-type. Oxidizing conditions result in decreased FKBP12.6 binding, but to the same extent as for the wild-type. Our findings suggest that FKBP12.6 regulation of RyR2 is unlikely to be the primary defect in inherited arrhythmogenic cardiac disease.

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