%0 Journal Article
%T Parkinson¡¯s disease protein DJ-1 regulates ATP synthase protein components to increase neuronal process outgrowth
%A Agnita J. W. Boon
%A Elizabeth A. Jonas
%A Giovanni Cossu
%A Han-A Park
%A Jack Tang
%A Jing Wu
%A Kambiz N. Alavian
%A Morven Graham
%A Nelli Mnatsakanyan
%A Paige Miranda
%A Pawel Licznerski
%A Peter J. S. Smith
%A Rongmin Chen
%A Vincenzo Bonifati
%A Wim Mandemakers
%A Yulong Niu
%J Archive of "Cell Death & Disease".
%D 2019
%R 10.1038/s41419-019-1679-x
%X a Coomassie staining of the protein bands present in submitochondrial vesicles (SMVs) is shown in the left panel. The right panel shows an immunoblot using an anti-DJ-1 antibody against solubilized protein from SMVs. The blot confirms that DJ-1 is present in the SMV subcellular fraction. b WT and mutant DJ-1 immunolocalize with ATP synthase ¦Â-subunit. Extracts from HEK293 cells expressing flag-tagged WT or mutant DJ-1 were prepared for coimmunoprecipitation analysis with anti-FLAG antibody. Proteins were revealed with the indicated antibodies in western blots. Note that endogenous ATP synthase ¦Â-subunit is copurified with WT DJ-1 and also with mutant DJ-1. c Purified WT and mutant DJ-1 immunolocalize with ATP synthase ¦Â-subunit in vitro. Purified flag-ATP synthase ¦Â-subunit protein was incubated with purified DJ-1. The mixture was immuno-precipitated with anti-DJ-1 antibody. The protein interaction was revealed by western blotting with the indicated antibodies. d The ability of SMVs to sequester H+ is improved by recombinant WT DJ-1 protein. Shown are ACMA (H+ indicator) fluorescence intensity changes induced in response to ATP in the presence of rat SMVs. A decrease in indicator intensity represents sequestration of H+ into the lumen of SMVs. Data were acquired every 30£¿s for 5£¿min in the presence of BSA control protein or recombinant protein (all proteins were added at 10£¿¦Ìg/100£¿¦Ìl final). For WT DJ-1 (n£¿=£¿6 samples), M3 DJ-1 (n£¿=£¿8) or M5 DJ-1 (n£¿=£¿8). WT DJ-1 protein maximizes the ability of SMVs to sequester H+ whereas mutant DJ-1 proteins have no effect. **p£¿<£¿0.01, *p£¿<£¿0.05, recombinant DJ-1 protein versus BSA control (n£¿=£¿6). Paired, two-tailed t-test. Error bars represent mean£¿¡À£¿SEM. e The ability of SMVs to sequester H+ is compromised in DJ-1£¿/£¿ SMVs. WT (n£¿=£¿7) and DJ-1£¿/£¿ (n£¿=£¿6). **p£¿<£¿0.01, *p£¿<£¿0.05, Error bars represent mean£¿¡À£¿SE
%K Mechanisms of disease
%K Diseases of the nervous system
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6565618/