Expression of a Cu,Zn superoxide dismutase typical of familial amyotrophic lateral sclerosis induces mitochondrial alteration and increase of cytosolic Ca2+ concentration in transfected neuroblastoma SH-SY5Y cells.

Abstract:

:We have set up a model system for familial amyotrophic lateral sclerosis (FALS) by transfecting human neuroblastoma cell line SH-SY5Y with plasmids directing constitutive expression of either wild-type human Cu,Zn superoxide dismutase (Cu,ZnSOD) or a mutant of this enzyme (G93A) associated with FALS. We have tested mitochondrial function and determined cytosolic Ca2+ concentration in control cells (untransfected) and in cells expressing either wild-type Cu,ZnSOD or G93A. We report that G93A induces a significant loss of mitochondrial membrane potential, an increased sensitivity toward valinomycin and a parallel increase in cytosolic Ca2+ concentration. The above phenomena are not related to total Cu,ZnSOD content and activity in the cell.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

Carrì MT,Ferri A,Battistoni A,Famhy L,Gabbianelli R,Poccia F,Rotilio G

doi

10.1016/s0014-5793(97)01051-x

subject

Has Abstract

pub_date

1997-09-08 00:00:00

pages

365-8

issue

2

eissn

0014-5793

issn

1873-3468

pii

S0014-5793(97)01051-X

journal_volume

414

pub_type

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