Definitions

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  • n. A metalloprotease associated with paraplegia

Etymologies

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Examples

  • P > 0.05. (b) Fluorescence immunoblot analysis and protein quantitation of mitochondria-encoded COX subunits Cox1p, Cox2p and Cox3p, and nuclear-encoded subunit Cox4p, in mitochondrial extracts from cells expressing AFG3L2 alone, in the absence of paraplegin.

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  • Along with paraplegin, which causes recessive spastic paraplegia, AFG3L2 is a component of the conserved m-AAA metalloprotease complex involved in the maintenance of the mitochondrial proteome.

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  • E691K and paraplegin (g), in which the positively charged Lys691 of AFG3L2

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  • E691K is not counteracted by the neutral Gln693 of paraplegin.

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  • Figure 6: Expression of AFG3L2 and paraplegin in human and mouse nervous systems.

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  • WT alone (lanes 2 and 7) or coexpressing either wild-type or mutant AFG3L2 with paraplegin

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  • We analyzed immunoprecipitates (IP) by SDS-PAGE and immunoblotting (IB) using anti-paraplegin (α-AFG3L2).

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  • AFG3L2 was detected in all the immunoprecipitates from yeast cells coexpressing paraplegin

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  • Substitutions affecting respiration are in bold. (a) COX activity measured in isolated mitochondria from yta10Δyta12Δ m-AAA-deficient yeast cells expressing AFG3L2 alone, in the absence of paraplegin.

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  • In addition, given our previous data, we quantified several mitochondrial protease genes, two that are part of the AAA proteases family and located in the inner membrane of the mitochondria (YME1L1 and SPG7 / paraplegin) and two located in the mitochondrial matrix (LON and CLPP).

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