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The mitochondrial transporter SFXN1 is critical for Complex III integrity and cellular metabolism

ABSTRACT: Mutations in acylglycerol kinase (AGK) leads to Sengers syndrome, a rare disease characterized by skeletal myopathy, hypertrophic cardiomyopathy, and cataracts. AGK, in vitro, produces lysophosphatidic acid and phosphatidic acid via phosphorylation of monoacylglycerol and diacylglycerol, accordingly. AGK is also a component of the TIM22 import machinery in the inner mitochondrial membrane and, independent of its kinase activity, has been shown to mediate the import of mitochondrial carrier family (SLC25) members. We compared the proteomes of mitochondria from HEK293 WT and AGK KO cells in order to identify additional candidate substrates of the TIM22 translocase that relies on AGK.

INSTRUMENT(S): Orbitrap Fusion Lumos

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Kidney Epithelium

SUBMITTER: Akhilesh Pandey

LAB HEAD: Steven Claypool

PROVIDER: PXD019826 | Pride | 2020-06-18

REPOSITORIES: Pride

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			Action	DRS
	Claypool_AGK_KO_SILAC_Set1_bRPLC_F01-24.msf	Msf
	Claypool_AGK_KO_SILAC_Set1_bRPLC_F01.raw	Raw
	Claypool_AGK_KO_SILAC_Set1_bRPLC_F02.raw	Raw
	Claypool_AGK_KO_SILAC_Set1_bRPLC_F03.raw	Raw
	Claypool_AGK_KO_SILAC_Set1_bRPLC_F04.raw	Raw

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