Supplementary Materials

Supplementary Materials for:

An RYR1 mutation associated with malignant hyperthermia is also associated with bleeding abnormalities

Rubén J. Lopez, Susan Byrne, Mirko Vukcevic, Marijana Sekulic-Jablanovic, Lifen Xu, Marijke Brink, Jay Alamelu, Nicol Voermans, Marc Snoeck, Emma Clement, Francesco Muntoni, Haiyan Zhou, Aleksandar Radunovic, Shehla Mohammed, Elizabeth Wraige, Francesco Zorzato, Susan Treves,* Heinz Jungbluth

*Corresponding author. Email: susan.treves{at}unibas.ch

This PDF file includes:

  • Fig. S1. RYR1Y522S female mice also exhibit prolonged bleeding times.
  • Fig. S2. Heart rate and systolic blood pressure are similar in wild-type and RYR1Y522S mice.
  • Fig. S3. Expression of RYR2 and RYR3 is not significantly different in the tail arteries of wild-type and RYR1Y522S mice.
  • Fig. S4. Specificity of the anti-RyR1 antibodies used for immunohistochemistry.
  • Fig. S5. Membrane potential measurements using the fluorescence potentiometric probe bis-oxonol.
  • Table S1. Detailed analysis of full kinetic parameters of sparks in vascular smooth muscle cells from wild-type and RYR1Y522S mice.
  • Legends for videos S1 to S5

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Other Supplementary Material for this manuscript includes the following:

  • Video S1 (.avi format). Sparks in smooth muscle cells from wild-type mice.
  • Video S2 (.avi format). Sparks in smooth muscle cells from RYR1Y522S mice.
  • Video S3 (.avi format). Sparks in smooth muscle cells from wild-type mice after treatment with 10 μM ryanodine.
  • Video S4 (.avi format). Sparks in smooth muscle cells from RYR1Y522S mice after treatment with 20 μM dantrolene.
  • VideoS5 (.avi format). Sparks in smooth muscle cells from RYR1Y522S mice after treatment with 250 nM xestospongin C.

Citation: R. J. Lopez, S. Byrne, M. Vukcevic, M. Sekulic-Jablanovic, L. Xu, M. Brink, J. Alamelu, N. Voermans, M. Snoeck, E. Clement, F. Muntoni, H. Zhou, A. Radunovic, S. Mohammed, E. Wraige, F. Zorzato, S. Treves, H. Jungbluth, An RYR1 mutation associated with malignant hyperthermia is also associated with bleeding abnormalities. Sci. Signal. 9, ra68 (2016).

© 2016 American Association for the Advancement of Science