%0 Journal Article %T Dysregulated calcium homeostasis prevents plasma membrane repair in Anoctamin 5/TMEM16E-deficient patient muscle cells %A Anant K. Menon %A Aurelia Defour %A Goutam Chandra %A Hiroki Morizono %A Ibrahim Mahjneh %A Jyoti K. Jaiswal %A Kalpana Pandey %A Kamel Mamchoui %A Mohammad Mahad Ahmad %A Nagarajan Pattabiraman %A SenChandra Sreetama %A Soumya Mishra %A Vincent Mouly %J Archive of "Cell Death Discovery". %D 2019 %R 10.1038/s41420-019-0197-z %X a Chromatogram for the sequence of the genomic DNA region of patient (middle and bottom) and healthy (top) myoblasts examining the mutation of the ANO5 gene. b Images showing immunostaining and (c) Quantification of the fluorescence intensity of ANO5 immunostaining in healthy (n£¿=£¿25 cells) and patient myoblasts (n£¿=£¿41 cells, ****p£¿<£¿0.0001). Scale bar£¿=£¿10£¿¦Ìm. d RT PCR quantification of ANO5 transcript using two independent ANO5 mRNA-specific primers. e Images showing myosin heavy chain 3 (MYH3, Green) and DAPI (nuclear, Red) staining of myoblasts differentiated for 10 days. Scale bar£¿=£¿10£¿¦Ìm. f Quantification of the number of myonuclei in healthy and patient myoblasts at indicated days following start of differentiation. g¨Cj Proteins isolated from differentiating myoblasts at indicated days post differentiation are analyzed by Western Blot for differentiation markers. g Representative Blots and h¨Cj Quantification of band intensity for proteins induced during differentiation h Myosin heavy chain 3 (MYH3), i Desmin and j ¦Á-actinin normalized to the loading control (¦Â-actin) (n£¿=£¿3 independent blots). The ¦Â-actin normalized band intensities were not significantly different between healthy and patient myoblasts based on the unpaired multiple t-tests except where indicated by *. (*p£¿=£¿0.046 for h and *p£¿=£¿0.049 for %K Neuromuscular disease %K Mechanisms of disease %U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6639303/