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Mmutations in paraplegin, a putative mitochondrial metallopeptidase of the AAA family, cause an autosomal recessive form of hereditary spastic paraplegia (HSP). Here, we analyze the function of paraplegin at the cellular level and characterize the phenotypic defects of HSP patients' cells lacking this protein. We demonstrate that paraplegin coassembles with(More)
The mitochondrial metalloprotease AFG3L2 assembles with the homologous protein paraplegin to form a supracomplex in charge of the essential protein quality control within mitochondria. Mutations of paraplegin cause a specific axonal degeneration of the upper motoneuron and, therefore, hereditary spastic paraplegia. Here we present two Afg3l2 murine models:(More)
The mitochondrial protein AFG3L2 forms homo-oligomeric and hetero-oligomeric complexes with paraplegin in the inner mitochondrial membrane, named m-AAA proteases. These complexes are in charge of quality control of misfolded proteins and participate in the regulation of OPA1 proteolytic cleavage, required for mitochondrial fusion. Mutations in AFG3L2 cause(More)
Resonant structures in B^{0}→ψ^{'}π^{-}K^{+} decays are analyzed by performing a four-dimensional fit of the decay amplitude, using pp collision data corresponding to 3  fb^{-1} collected with the LHCb detector. The data cannot be described with K^{+}π^{-} resonances alone, which is confirmed with a model-independent approach. A highly significant(More)
A measurement of the ratio of the branching fractions of the B(+) → K(+)μ(+)μ(-) and B(+) → K(+)e(+)e(-) decays is presented using proton-proton collision data, corresponding to an integrated luminosity of 3.0 fb(-1), recorded with the LHCb experiment at center-of-mass energies of 7 and 8 TeV. The value of the ratio of branching fractions for the dilepton(More)
The first full amplitude analysis of B^{+}→J/ψϕK^{+} with J/ψ→μ^{+}μ^{-}, ϕ→K^{+}K^{-} decays is performed with a data sample of 3  fb^{-1} of pp collision data collected at sqrt[s]=7 and 8 TeV with the LHCb detector. The data cannot be described by a model that contains only excited kaon states decaying into ϕK^{+}, and four J/ψϕ structures are observed,(More)
Measurements of the cross section for producing b quarks in the reaction pp→bb[over ¯]X are reported in 7 and 13 TeV collisions at the LHC as a function of the pseudorapidity η in the range 2<η<5 covered by the acceptance of the LHCb experiment. The measurements are done using semileptonic decays of b-flavored hadrons decaying into a ground-state charmed(More)
Charm meson oscillations are observed in a time-dependent analysis of the ratio of D^{0}→K^{+}π^{-}π^{+}π^{-} to D^{0}→K^{-}π^{+}π^{-}π^{+} decay rates, using data corresponding to an integrated luminosity of 3.0  fb^{-1} recorded by the LHCb experiment. The measurements presented are sensitive to the phase-space averaged ratio of doubly Cabibbo-suppressed(More)
The data sample of Λ_{b}^{0}→J/ψpK^{-} decays acquired with the LHCb detector from 7 and 8 TeV pp collisions, corresponding to an integrated luminosity of 3  fb^{-1}, is inspected for the presence of J/ψp or J/ψK^{-} contributions with minimal assumptions about K^{-}p contributions. It is demonstrated at more than nine standard deviations that(More)
The CP asymmetry in the mixing of B_{s}^{0} and B[over ¯]_{s}^{0} mesons is measured in proton-proton collision data corresponding to an integrated luminosity of 3.0  fb^{-1}, recorded by the LHCb experiment at center-of-mass energies of 7 and 8 TeV. Semileptonic B_{s}^{0} and B[over ¯]_{s}^{0} decays are studied in the inclusive mode D_{s}^{∓}μ^{±}ν[over(More)