NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4

Autor:	Merel J W, Adjobo-Hermans, Ria, de Haas, Peter H G M, Willems, Aleksandra, Wojtala, Sjenet E, van Emst-de Vries, Jori A, Wagenaars, Mariel, van den Brand, Richard J, Rodenburg, Jan A M, Smeitink, Leo G, Nijtmans, Leonid A, Sazanov, Mariusz R, Wieckowski, Werner J H, Koopman
Rok vydání:	2020
Předmět:	Mitochondrial Proteins Gene Knockout Techniques Mice Electron Transport Complex I Protein Stability NADPH Dehydrogenase Animals Humans Fibroblasts Leigh Disease Gene Deletion Oxidative Phosphorylation Molecular Chaperones
Zdroj:	Biochimica et biophysica acta. Bioenergetics. 1861(8)
ISSN:	1879-2650
Popis:	Mutations in NDUFS4, which encodes an accessory subunit of mitochondrial oxidative phosphorylation (OXPHOS) complex I (CI), induce Leigh syndrome (LS). LS is a poorly understood pediatric disorder featuring brain-specific anomalies and early death. To study the LS pathomechanism, we here compared OXPHOS proteomes between various Ndufs4
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=pmid________::17f057b315e2d62d5f878aaa0c00d4c5 https://pubmed.ncbi.nlm.nih.gov/32335026 Zobrazit plný text záznamu Full Text from ScienceDirect