H2020Индивидуална стипендия2015–2017

MitoTAGs · Developing next-generation tools for mitochondrial dissection with cell-specific resolution.

„Хоризонт 2020“ — Действия „Мария Склодовска-Кюри“

Период: 2015-05-01 → 2017-04-30
Финансиране от ЕС: 170 122 €
Участници: 1
Схема: MSCA-IF-EF-ST

Линиите свързват координатора с партньорите.

Накратко на български

Митохондриите в различните неврони се изследват, за да се разбере защо някои клетки в мозъка са по-податливи на заболявания от други. Това помага за откриването на нови терапевтични мишени при първични митохондриални болести, диабет и невродегенерация.

Този кратък обзор е генериран от изкуствен интелект

Кратко обяснение, генерирано от езиков модел по текста на CORDIS. Оригиналът е по-долу.

Резултати накратко

Developing next-generation tools for mitochondrial dissection with cell-specific resolution.

Severe alterations of the mitochondrial machinery involved in energy generation lead to a group of progressive and usually fatal pathologies collectively known as primary mitochondrial disease (MD), affecting 1:5000 births. Currently, there is no cure and the treatments available are mostly ineffective. MD predominantly affects high energy-requiring organs such as the brain. However, not all neurons are equally vulnerable to MD, but rather show a striking anatomical and cellular specificity. The mechanisms conferring neuronal resistance or vulnerability to MD are currently unknown. To date, research in mitochondrial disease has been hindered by the high degree of variability in disease progression and severity in human patients, and the intrinsic heterogeneity of mitochondria. Furthermore, model systems both in vivo and in vitro to date have failed to identify the mechanisms underlying the anatomical and cellular specificity of these pathologies. Hence, if one could possess the ability to identify, purify and compare cellular and mitochondrial changes from affected and healthy neurons, a better and more significant insight on the biochemical and functional changes associated with MD would be obtained. To address this issue, two ground-breaking approaches were developed to define the molecular basis of neuronal susceptibility to mitochondrial disease. These approaches have the potential to propose new therapeutic targets for MD and are easily applicable to other pathologies associated with mitochondrial dysfunction such as diabetes or neurodegenerative processes.

Текст от CORDIS, на английски · Данни: CORDIS, © Европейски съюз

Цел на проекта

Mitochondria generate most of the energy cells require to function. Deficits in the mitochondrial energy-generating machinery affect 1:5,000 children and cause progressive, debilitating, and usually fatal pathologies collectively known as primary mitochondrial disease. To date, there is no cure for mitochondrial disease and existing treatments are highly ineffective and mostly palliative. High-energy-requiring cells, such as neurons, are especially affected in mitochondrial disease. However, not all neuronal populations are equally affected. Furthermore, the molecular determinants of neuronal vulnerability to mitochondrial disease have not been adequately elucidated, representing a challenge for the development of efficient treatments for these pathologies. To improve on current knowledge on mitochondrial disease and to provide better therapeutic targets, this proposal focuses on developing ground-breaking molecular biology tools that will allow the identification and dissection of the molecular determinants of neuronal vulnerability in mitochondrial disease with unprecedented definition. I will develop novel techniques to isolate the mitochondrial translatome by using ribosomal tagging, as well as to assess intact mitochondrial function, with cell-type specificity. These novel approaches will have a high impact in mitochondrial disease research, with the overall aim of identifying novel therapeutic targets that will lead to effective treatments for mitochondrial disease. Furthermore, the high applicability of the tools generated will allow significant breakthroughs in the research of other pathologies with mitochondrial affectation such as diabetes or neurodegenerative processes.

Оригинален текст от CORDIS (на английски).

Участници

UNIVERSITAT AUTONOMA DE BARCELONA · Cerdanyola Del VallesКоординаторИспания