Structure-function analysis of mitochondrial alpha-keto acid dehydrogenase complex mutations causing nervous system impairment

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Details of project

Identifier

143627

Type

Principal investigator

Ambrus, Attila

Title in Hungarian

Idegrendszeri károsodást okozó mitokondriális alfa-ketosav-dehidrogenáz enzimkomplex mutációk szerkezet-funkció analízise

Title in English

Structure-function analysis of mitochondrial alpha-keto acid dehydrogenase complex mutations causing nervous system impairment

Keywords in Hungarian

molekuláris patomechanizmus; patogén mutáció; reaktív oxigénszármazék; lipoamid-dehidrogenáz; alfa-ketosav-dehidrogenázok; mitokondrium; röntgenkrisztallográfia; krio-EM; bioenergetika

Keywords in English

molecular pathomechanism; pathogenic mutation; reactive oxygen species; lipoamide dehydrogenase; alpha-keto acid dehydrogenases; mitochondrion; x-ray crystalography; cryo-EM; bioenergetics

Discipline

General biochemistry and metabolism (Council of Medical and Biological Sciences)	50 %
Structural biology (crystallography and EM) (Council of Medical and Biological Sciences)	50 %

Panel

Molecular and Structural Biology and Biochemistry

Department or equivalent

Dept. of Medical Biochemistry (Semmelweis University)

Participants

Komlódi, Tímea
Nagy, Bálint
Ozohanics, Oliver
Sváb, Gergely
Szabó, Eszter
Tretter, László
Vass, Krisztina Rubina
Zámbó, Zsófia

Starting date

2022-09-01

Closing date

2026-08-31

Funding (in million HUF)

48.000