Eps 15 Homology Domain (EHD)-1 Remodels Transverse Tubules in Skeletal Muscle

PloS One
Alexis R DemonbreunElizabeth M McNally

Abstract

We previously showed that Eps15 homology domain-containing 1 (EHD1) interacts with ferlin proteins to regulate endocytic recycling. Myoblasts from Ehd1-null mice were found to have defective recycling, myoblast fusion, and consequently smaller muscles. When expressed in C2C12 cells, an ATPase dead-EHD1 was found to interfere with BIN1/amphiphysin 2. We now extended those findings by examining Ehd1-heterozygous mice since these mice survive to maturity in normal Mendelian numbers and provide a ready source of mature muscle. We found that heterozygosity of EHD1 was sufficient to produce ectopic and excessive T-tubules, including large intracellular aggregates that contained BIN1. The disorganized T-tubule structures in Ehd1-heterozygous muscle were accompanied by marked elevation of the T-tubule-associated protein DHPR and reduction of the triad linker protein junctophilin 2, reflecting defective triads. Consistent with this, Ehd1-heterozygous muscle had reduced force production. Introduction of ATPase dead-EHD1 into mature muscle fibers was sufficient to induce ectopic T-tubule formation, seen as large BIN1 positive structures throughout the muscle. Ehd1-heterozygous mice were found to have strikingly elevated serum creatine kin...Continue Reading

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Citations

Dec 8, 2017·The Journal of Immunology : Official Journal of the American Association of Immunologists·Fany M IsekaHamid Band
Sep 15, 2019·Journal of Muscle Research and Cell Motility·Ashraf KitmittoElizabeth J Cartwright
Mar 31, 2020·Protein Science : a Publication of the Protein Society·Soumya Bhattacharyya, Thomas J Pucadyil
Sep 24, 2019·The Journal of Clinical Investigation·Alexis R DemonbreunElizabeth M McNally
Nov 12, 2018·Developmental Biology·Chih-Ning ChangChrissa Kioussi

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Methods Mentioned

BETA
GTPases
biopsies
force measurements
Assay

Software Mentioned

Prism
Image J
Fiji
Clampfit

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