Show simple item record

dc.contributor.authorYeo, AJ
dc.contributor.authorChong, KL
dc.contributor.authorGatei, M
dc.contributor.authorZou, D
dc.contributor.authorStewart, R
dc.contributor.authorWithey, S
dc.contributor.authorWolvetang, E
dc.contributor.authorParton, RG
dc.contributor.authorBrown, AD
dc.contributor.authorKastan, MB
dc.contributor.authorComan, D
dc.contributor.authorLavin, MF
dc.date.accessioned2021-01-13T05:41:46Z
dc.date.available2021-01-13T05:41:46Z
dc.date.issued2021
dc.identifier.issn2589-0042en_US
dc.identifier.doi10.1016/j.isci.2020.101972en_US
dc.identifier.urihttp://hdl.handle.net/10072/401006
dc.description.abstractThere is evidence that ATM mutated in ataxia-telangiectasia (A-T) plays a key role in protecting against mitochondrial dysfunction, the mechanism for which remains unresolved. We demonstrate here that ATM-deficient cells are exquisitely sensitive to nutrient deprivation, which can be explained by defective cross talk between the endoplasmic reticulum (ER) and the mitochondrion. Tethering between these two organelles in response to stress was reduced in cells lacking ATM, and consistent with this, Ca2+ release and transfer between ER and mitochondria was reduced dramatically when compared with control cells. The impact of this on mitochondrial function was evident from an increase in oxygen consumption rates and a defect in mitophagy in ATM-deficient cells. Our findings reveal that ER-mitochondrial connectivity through IP3R1-GRP75-VDAC1, to maintain Ca2+ homeostasis, as well as an abnormality in mitochondrial fusion defective in response to nutrient stress, can account for at least part of the mitochondrial dysfunction observed in A-T cells.en_US
dc.description.peerreviewedYesen_US
dc.languageEnglish
dc.language.isoeng
dc.publisherElsevier
dc.relation.ispartofpagefrom101972en_US
dc.relation.ispartofissue1en_US
dc.relation.ispartofjournaliScienceen_US
dc.relation.ispartofvolume24en_US
dc.subject.fieldofresearchClinical Sciencesen_US
dc.subject.fieldofresearchcode1103en_US
dc.titleImpaired endoplasmic reticulum-mitochondrial signaling in ataxia-telangiectasiaen_US
dc.typeJournal articleen_US
dc.type.descriptionC1 - Articlesen_US
dcterms.bibliographicCitationYeo, AJ; Chong, KL; Gatei, M; Zou, D; Stewart, R; Withey, S; Wolvetang, E; Parton, RG; Brown, AD; Kastan, MB; Coman, D; Lavin, MF, Impaired endoplasmic reticulum-mitochondrial signaling in ataxia-telangiectasia, iScience, 2021, 24 (1), pp. 101972en_US
dcterms.licensehttp://creativecommons.org/licenses/by-nc-nd/4.0/en_US
dc.date.updated2021-01-13T05:36:48Z
dc.description.versionVersion of Record (VoR)en_US
gro.rights.copyright© 2020 The Authors. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).en_US
gro.hasfulltextFull Text
gro.griffith.authorComan, Dave J.


Files in this item

This item appears in the following Collection(s)

  • Journal articles
    Contains articles published by Griffith authors in scholarly journals.

Show simple item record