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The biochemical basis of mitochondrial dysfunction in Zellweger Spectrum Disorder

ABSTRACT: We identified that peroxins were still expressed in Zellweger Spectrum Disorder (ZSD) and a subset of them accumulated on the mitochondrial membrane, which resulted in gross mitochondrial abnormalities and impaired mitochondrial metabolic function. In this complexome analysis we detected several peroxins in the mitochondrial fraction in the absence of functional peroxisomes and that this accumulation is exacerbated by the loss of the mitochondrial extractase Msp1. In addition, we observed that specific peroxisomal matrix proteins localize to the mitochondria, which suggest that a functional peroxisomal docking and import complex assembles on the mitochondria in the absence of peroxisomes.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Saccharomyces Cerevisiae (baker's Yeast)

TISSUE(S): Cell Culture

SUBMITTER: Ilka Wittig

LAB HEAD: Jared Rutter

PROVIDER: PXD024625 | Pride | 2021-06-25

REPOSITORIES: Pride

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			Action	DRS
	Complexomes.xlsx	Xlsx
	Gel.jpg	Other
	P18_011_Esther_c_mitos_245_01.raw	Raw
	P18_011_Esther_c_mitos_245_02.raw	Raw
	P18_011_Esther_c_mitos_245_03.raw	Raw

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