NDLI: Respiratory Complex I in Brain Development and Genetic Disease

Content Provider	Springer Nature Link
Author	Papa, Sergio Petruzzella, Vittoria Scacco, Salvatore Vergari, Rosaria Panelli, Damia Tamborra, Rosanna Corsi, Patrizia Picciariello, Margherita Lambo, Rossana Bertini, Enrico Santorelli, Filippo Maria
Copyright Year	2004
Abstract	A study is presented on the expression and activity of complex I, as well as of other complexes of the respiratory chain, in the course of brain development and inherited encephalopathies. Investigations on mouse hippocampal cells show that differentiation of these cells both in vivo and in cell cultures is associated with the expression of a functional complex I, whose activity markedly increases with respect to that of complexes III and IV. Data are presented on genetic defects of complex I in six children with inborn encephalopathy associated with isolated deficiency of the complex. Mutations have been identified in nuclear and mitochondrial genes coding for subunits of the complex. Different mutations were found in the nuclear NDUFS4 gene coding for the 18 kD (IP, AQDQ) subunit of complex I. All the NDUFS4 mutations resulted in impairment of the assembly of a functional complex. The observations presented provide evidence showing a critical role of complex I in differentiation and functional activity of brain cells.
Starting Page	547
Ending Page	560
Page Count	14
File Format	PDF
ISSN	03643190
Journal	Neurochemical Research
Volume Number	29
Issue Number	3
e-ISSN	15736903
Language	English
Publisher	Kluwer Academic Publishers-Plenum Publishers
Publisher Date	2004-01-01
Publisher Place	New York
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Neurosciences Neurology Biochemistry
Content Type	Text
Resource Type	Article
Subject	Medicine Biochemistry Cellular and Molecular Neuroscience

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