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Insights into the dynamics of the ATP-dependent human mitochondrial Lon protease by its three-dimensional electron microscopic structural analysis

Project goals

Lon proteases are the most flexible ATP-dependent proteases, which are both in prokaryotic and eukaryotic cells responsible for protein degradation, regulate levels of transcription factors and supposedly participate in apoptosis. Malfunctions of these proteases are linked with dystrophies, neurodegenerative diseases and cancer. A deeper insight into their mechanisms of action requires detailed knowledge of their native three-dimensional (3D) structure in the course of its main functional cycle, the ATP-ase cycle. 3D structure of any flexible Lon protease complex has not been determined so far, however, human mitochondrial Lon protease is the most stable Lon protease purified so far, and therefore we will employ conventional and cryo-electron microscopy in connection with the single particle analysis method to determine the 3D structure of the wild-type of human Lon in the course of its ATP-ase cycle, i.e. in interactions with Mg2+ cations, ATP, ADP and protein substrate, as well as of a few of its available mutant forms, with expected resolutions of ~1nm.

Keywords

Human mitochondrial Lon proteasemitochondria(cryo-) transmission electron microscopysingle particle analysis3D reconstruction

Public support

  • Provider

    Czech Science Foundation

  • Programme

    Post-graduate (doctorate) grants

  • Call for proposals

    Postdoktorandské granty 14 (SGA0201300006)

  • Main participants

    Univerzita Karlova / 1. lékařská fakulta

  • Contest type

    VS - Public tender

  • Contract ID

    13-32339P

Alternative language

  • Project name in Czech

    Pohled do dynamiky ATP-dependentní lidské mitochondriální Lon proteázy trojrozměrnou elektronově mikroskopickou strukturní analýzou

  • Annotation in Czech

    Lon proteázy jsou strukturně nejflexibilnější ATP-závislé proteázy, jež jsou v buňkách prokaryot i eukaryot zodpovědné za degradaci poškozených proteinů, udržují hladinu krátkodobě existujících bílkovin a předpokládá se jejich účast v apoptóze. Poruchy ve funkci těchto proteáz jsou spojovány s dystrofiemi, neurodgenerativními onemocněními a rakovinou. Hlubší porozumění jejich funkčním mechanismům vyžaduje znalost jejich nativní trojrozměrné (3D) struktury v průběhu jejich hlavního funkčního cyklu, tzv. ATP-cyklu. 3D strukturu žádné Lon proteázy se však zatím nepodařilo určit, lidská mitochondriální Lon proteáza je však dosud nejstabilnější izolovanou Lon proteázou, a proto konvenční a kryo-elektronovou mikroskopií ve spojení s metodami single-particle analýzy (SPA) určíme strukturu wild-type Lon v průběhu jejího ATP-cyklu, tj. při interakci s kationty Mg2+, ATP, ADP a proteinovým substrátem, a strukturu několika mutantních forem s předpokládaným rozlišením ~1 nm.

Scientific branches

  • R&D category

    ZV - Basic research

  • CEP classification - main branch

    EB - Genetics and molecular biology

  • CEP - secondary branch

    BO - Biophysics

  • CEP - another secondary branch

  • 10603 - Genetics and heredity (medical genetics to be 3)
    10604 - Reproductive biology (medical aspects to be 3)
    10605 - Developmental biology
    10608 - Biochemistry and molecular biology
    10609 - Biochemical research methods
    10610 - Biophysics
    30101 - Human genetics

Completed project evaluation

  • Provider evaluation

    U - Uspěl podle zadání (s publikovanými či patentovanými výsledky atd.)

  • Project results evaluation

    The results of this three-year postdoctoral project enabled to understand the function of Lon protease, which when expressed more than usual is sensed as a marker of tumour diseases. Received structural information are new and can shift forward the future research on this enzyme. Four publications are dedicated on this project.

Solution timeline

  • Realization period - beginning

    Feb 1, 2013

  • Realization period - end

    Nov 24, 2016

  • Project status

    U - Finished project

  • Latest support payment

    Apr 1, 2016

Data delivery to CEP

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

  • Data delivery code

    CEP17-GA0-GP-U/01:1

  • Data delivery date

    Jun 30, 2017

Finance

  • Total approved costs

    2,835 thou. CZK

  • Public financial support

    2,835 thou. CZK

  • Other public sources

    0 thou. CZK

  • Non public and foreign sources

    0 thou. CZK