Dataset Information

Diverse mitochondrial abnormalities in a new cellular model of TAFFAZZIN deficiency are remediated by Cardiolipin-interacting small molecules

ABSTRACT: With the goal of identifying unappreciated pathways of mitochondrial dysregulation in Barth Syndrome, we utilized an unbiased proteomics strategy to identify pathways of metabolic dysregulation in an HEK293-based TAZ-deficiency model.

INSTRUMENT(S): Orbitrap Fusion

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Hilary Vernon

LAB HEAD: Hillary Vernon

PROVIDER: PXD026731 | Pride | 2022-02-17

REPOSITORIES: Pride

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Dataset's files

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			Action	DRS
	SK-CS-171215_VernonH_AF_F.mgf	Mgf
	SK-CS-171215_VernonH_AF_F.mzML	Mzml
	SK-CS-171215_VernonH_AF_F.mzid.gz	Mzid
	SK-CS-171215_VernonH_AF_F1.raw	Raw
	SK-CS-171215_VernonH_AF_F10.raw	Raw

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Diverse mitochondrial abnormalities in a new cellular model of TAFFAZZIN deficiency are remediated by cardiolipin-interacting small molecules.

Anzmann Arianna F AF Sniezek Olivia L OL Pado Alexandra A Busa Veronica V Vaz Frédéric M FM Kreimer Simion D SD DeVine Lauren R LR Cole Robert N RN Le Anne A Kirsch Brian J BJ Claypool Steven M SM Vernon Hilary J HJ

The Journal of biological chemistry 20210724 3

Barth syndrome (BTHS) is an X-linked disorder of mitochondrial phospholipid metabolism caused by pathogenic variants in TAFFAZIN, which results in abnormal cardiolipin (CL) content in the inner mitochondrial membrane. To identify unappreciated pathways of mitochondrial dysfunction in BTHS, we utilized an unbiased proteomics strategy and identified that complex I (CI) of the mitochondrial respiratory chain and the mitochondrial quality control protease presenilin-associated rhomboid-like protein ...[more]

PMID: 34314685

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