Nuclear-encoded mitochondrial complex I gene expression is restored to normal levels by inhibition of unedited ATP9 transgene expression in Arabidopsis thaliana

2006 ◽  
Vol 44 (1) ◽  
pp. 1-6 ◽  
Author(s):  
María V. Busi ◽  
Diego F. Gómez-Casati ◽  
Mariano Perales ◽  
Alejandro Araya ◽  
Eduardo Zabaleta
Keyword(s):  
Gene Expression ◽  
Arabidopsis Thaliana ◽  
Complex I ◽  
Transgene Expression ◽  
Mitochondrial Complex I ◽  
Mitochondrial Complex ◽  
I Gene

Mitochondrial complex I gene mutations drive metabolic reprogramming in prostate cancer

2019 ◽  
Vol 18 (8) ◽  
pp. e3041
Author(s):  
B. Schöpf ◽  
H. Weissensteiner ◽  
G. Schäfer ◽  
A. Naschberger ◽  
B. Rupp ◽  
...  
Keyword(s):  
Prostate Cancer ◽  
Complex I ◽  
Gene Mutations ◽  
Metabolic Reprogramming ◽  
Mitochondrial Complex I ◽  
Mitochondrial Complex ◽  
I Gene

scholarly journals A ferredoxin bridge connects the two arms of plant mitochondrial complex I

2020 ◽  
Author(s):  
Niklas Klusch ◽  
Jennifer Senkler ◽  
Özkan Yildiz ◽  
Werner Kühlbrandt ◽  
Hans-Peter Braun
Keyword(s):  
Arabidopsis Thaliana ◽  
Complex I ◽  
Mitochondrial Membrane ◽  
Mitochondrial Complex I ◽  
Mitochondrial Complex ◽  
Inner Mitochondrial Membrane ◽  
Main Site ◽  
The Matrix ◽  
Quinol Binding ◽  
Complex I Activity

SUMMARYMitochondrial complex I is the main site for electron transfer to the respiratory chain and generates much of the proton gradient across the inner mitochondrial membrane. It is composed of two arms, which form a conserved L-shape. We report the structures of the intact, 47-subunit mitochondrial complex I from Arabidopsis thaliana and from the green alga Polytomella sp. at 3.2 and 3.3 Å resolution. In both, a heterotrimeric γ-carbonic anhydrase domain is attached to the membrane arm on the matrix side. Two states are resolved in A. thaliana complex I, with different angles between the two arms and different conformations of the ND1 loop near the quinol binding site. The angle appears to depend on a bridge domain, which links the peripheral arm to the membrane arm and includes an unusual ferredoxin. We suggest that the bridge domain regulates complex I activity.One sentence summaryThe activity of complex I depends on the angel between its two arms, which, in plants, is adjusted by a protein bridge that includes an unusual ferredoxin.The authors responsible for distribution of materials integral to the findings presented in this article in accordance with the policy described in the Instructions for Authors (www.plantcell.org) are: Hans-Peter Braun ([email protected]) and Werner Kühlbrandt ([email protected]).

scholarly journals Maternally Inherited Differences within Mitochondrial Complex I Control Murine Healthspan

Genes ◽  
2019 ◽  
Vol 10 (7) ◽  
pp. 532
Author(s):  
Misa Hirose ◽  
Paul Schilf ◽  
Kim Zarse ◽  
Hauke Busch ◽  
Georg Fuellen ◽  
...  
Keyword(s):  
Experimental Evidence ◽  
Complex I ◽  
Association Studies ◽  
Mitochondrial Complex I ◽  
Enzyme Complex ◽  
Mitochondrial Complex ◽  
Age Related ◽  
Functional Relevance ◽  
Invertebrate Models ◽  
I Gene

Mitochondrial complex I—the largest enzyme complex of the mitochondrial oxidative phosphorylation machinery—has been proposed to contribute to a variety of age-related pathological alterations as well as longevity. The enzyme complex-consisting proteins are encoded by both nuclear (nDNA) and mitochondrial DNA (mtDNA). While some association studies of mtDNA encoded complex I genes and lifespan in humans have been reported, experimental evidence and the functional consequence of such variants is limited to studies using invertebrate models. Here, we present experimental evidence that a homoplasmic mutation in the mitochondrially encoded complex I gene mt-Nd2 modulates lifespan by altering cellular tryptophan levels and, consequently, ageing-related pathways in mice. A conplastic mouse strain carrying a mutation at m.4738C > A in mt-Nd2 lived slightly, but significantly, shorter than the controls did. The same mutation led to a higher susceptibility to glucose intolerance induced by high-fat diet feeding. These phenotypes were not observed in mice carrying a mutation in another mtDNA encoded complex I gene, mt-Nd5, suggesting the functional relevance of particular mutations in complex I to ageing and age-related diseases.

scholarly journals Maternally inherited differences within mitochondrial Complex I control murine healthspan

2018 ◽  
Author(s):  
Misa Hirose ◽  
Paul Schilf ◽  
Kim Zarse ◽  
Hauke Busch ◽  
Georg Füllen ◽  
...  
Keyword(s):  
Experimental Evidence ◽  
Complex I ◽  
Association Studies ◽  
Mitochondrial Complex I ◽  
Enzyme Complex ◽  
Mitochondrial Complex ◽  
Age Related ◽  
Functional Relevance ◽  
Invertebrate Models ◽  
I Gene

AbstractMitochondrial complex I, the largest enzyme complex of the mitochondrial oxidative phosphorylation machinery, has been proposed to contribute to a variety of age-related pathological alterations as well as longevity. The enzyme complex-consisting proteins are encoded by both nuclear (nDNA) and mitochondrial DNA (mtDNA). While some association studies of mtDNA-encoded complex I genes and lifespan in humans have been reported, experimental evidence and the functional consequence of such variants is limited to studies using invertebrate models. Here, we present experimental evidence that a homoplasmic mutation in the mitochondrially encoded complex I gene mt-Nd2 modulates lifespan by altering cellular tryptophan levels and, consequently, ageing-related pathways in mice. A conplastic mouse strain carrying a mutation at m.4738C>A in mt-Nd2 lived significantly shorter than the controls did. The same mutation led to a higher susceptibility to glucose intolerance induced by high-fat diet feeding. These phenotypes were not observed in mice carrying a mutation in another mtDNA-encoded complex I gene, mt-Nd5, suggesting the functional relevance of particular mutations in complex I to ageing and age-related diseases.

A heteroplasmic mitochondrial complex I gene mutation in adult-onset dystonia

Neurogenetics ◽  
2003 ◽  
Vol 4 (4) ◽  
pp. 199-205 ◽  
Author(s):  
DavidK. Simon ◽  
Jennifer Friedman ◽  
XandraO. Breakefield ◽  
Joseph Jankovic ◽  
MitchellF. Brin ◽  
...  
Keyword(s):  
Gene Mutation ◽  
Complex I ◽  
Mitochondrial Complex I ◽  
Adult Onset ◽  
Mitochondrial Complex ◽  
I Gene

scholarly journals A frameshift mitochondrial complex I gene mutation in a patient with dystonia and cataracts: is the mutation pathogenic?

2001 ◽  
Vol 38 (1) ◽  
pp. 58-61 ◽  
Author(s):  
D. K. SIMON ◽  
M. A. TARNOPOLSKY ◽  
J. T. GREENAMYRE ◽  
D. R. JOHNS
Keyword(s):  
Gene Mutation ◽  
Complex I ◽  
Mitochondrial Complex I ◽  
Mitochondrial Complex ◽  
I Gene

Gene expression study of mitochondrial complex I in schizophrenia and paranoid personality disorder

2017 ◽  
Vol 19 (sup3) ◽  
pp. S133-S146 ◽  
Author(s):  
Arvin Haghighatfard ◽  
Sarah Andalib ◽  
Mozhdeh Amini Faskhodi ◽  
Soha Sadeghi ◽  
Amir Hossein Ghaderi ◽  
...  
Keyword(s):  
Gene Expression ◽  
Personality Disorder ◽  
Complex I ◽  
Mitochondrial Complex I ◽  
Gene Expression Study ◽  
Mitochondrial Complex ◽  
Expression Study ◽  
Paranoid Personality Disorder ◽  
Paranoid Personality

scholarly journals Internal Architecture of Mitochondrial Complex I from Arabidopsis thaliana

2010 ◽  
Vol 22 (3) ◽  
pp. 797-810 ◽  
Author(s):  
Jennifer Klodmann ◽  
Stephanie Sunderhaus ◽  
Manfred Nimtz ◽  
Lothar Jänsch ◽  
Hans-Peter Braun
Keyword(s):  
Arabidopsis Thaliana ◽  
Complex I ◽  
Mitochondrial Complex I ◽  
Mitochondrial Complex ◽  
Internal Architecture

scholarly journals A Mitochondrial Complex I Defect Impairs Cold-Regulated Nuclear Gene Expression

2002 ◽  
Vol 14 (6) ◽  
pp. 1235-1251 ◽  
Author(s):  
Byeong-ha Lee ◽  
Hojoung Lee ◽  
Liming Xiong ◽  
Jian-Kang Zhu
Keyword(s):  
Gene Expression ◽  
Complex I ◽  
Nuclear Gene ◽  
Mitochondrial Complex I ◽  
Mitochondrial Complex ◽  
Nuclear Gene Expression
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