Lithium Sensitivity of Store Operated Ca2+ Entry and Survival of Fibroblasts Isolated from Chorea-Acanthocytosis Patients | Zendy

Lisann Pelzl | Zendy; Bhaeldin Elsir | Zendy; Itishri Sahu | Zendy; Rosi Bissinger | Zendy; Yogesh Singh | Zendy; Basma Sukkar | Zendy; Sabina Honisch | Zendy; Ludger Schoels | Zendy; Mohamed Jèmaà | Zendy; Elisabeth Lang | Zendy; Alexander Storch | Zendy; Andreas Hermann | Zendy; Christos Stournaras | Zendy; Florian Läng | Zendy

Open Access

Lithium Sensitivity of Store Operated Ca2+ Entry and Survival of Fibroblasts Isolated from Chorea-Acanthocytosis Patients

Author(s) -

Lisann Pelzl,

Bhaeldin Elsir,

Itishri Sahu,

Rosi Bissinger,

Yogesh Singh,

Basma Sukkar,

Sabina Honisch,

Ludger Schoels,

Mohamed Jèmaà,

Elisabeth Lang,

Alexander Storch,

Andreas Hermann,

Christos Stournaras,

Florian Läng

Publication year - 2017

Publication title -

cellular physiology and biochemistry

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.486

H-Index - 87

eISSN - 1421-9778

pISSN - 1015-8987

DOI - 10.1159/000479901

Subject(s) - propidium iodide , annexin , thapsigargin , chemistry , apoptosis , medicine , endocrinology , microbiology and biotechnology , programmed cell death , biology , biochemistry , endoplasmic reticulum

The widely expressed protein chorein fosters activation of the phosphoinositide 3 kinase (PI3K) pathway thus supporting cell survival. Loss of function mutations of the chorein encoding gene VPS13A (vacuolar protein sorting-associated protein 13A) causes chorea-acanthocytosis (ChAc), a neurodegenerative disorder paralleled by deformations of erythrocytes. In mice, genetic knockout of chorein leads to enhanced neuronal apoptosis. PI3K dependent signalling upregulates Orai1, a pore forming channel protein accomplishing store operated Ca2+ entry (SOCE). Increased Orai1 expression and SOCE have been shown to confer survival of tumor cells. SOCE could be up-regulated by lithium. The present study explored, whether SOCE and/or apoptosis are altered in ChAc fibroblasts and could be modified by lithium treatment.

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