Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease
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J. Silke | M. Lazarou | D. Thorburn | C. Sugiana | M. McKenzie | A. Connelly | J. Fletcher | Cjr Dunning | Dm Kirby | Mt Ryan
[1] N. Pfanner,et al. The protein import machinery of mitochondria , 2007 .
[2] Rutger O. Vogel,et al. Investigation of the complex I assembly chaperones B17.2L and NDUFAF1 in a cohort of CI deficient patients. , 2007, Molecular genetics and metabolism.
[3] M. Lazarou,et al. Analysis of mitochondrial subunit assembly into respiratory chain complexes using Blue Native polyacrylamide gel electrophoresis. , 2007, Analytical biochemistry.
[4] M. Lazarou,et al. Analysis of the Assembly Profiles for Mitochondrial- and Nuclear-DNA-Encoded Subunits into Complex I , 2007, Molecular and Cellular Biology.
[5] Rutger O. Vogel,et al. Cytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assembly. , 2007, Genes & development.
[6] F. Fontanesi,et al. Assembly of mitochondrial cytochrome c-oxidase, a complicated and highly regulated cellular process. , 2006, American journal of physiology. Cell physiology.
[7] John E. Walker,et al. Bovine Complex I Is a Complex of 45 Different Subunits* , 2006, Journal of Biological Chemistry.
[8] M. Lazarou,et al. Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients. , 2006, Journal of molecular biology.
[9] H. Fukui,et al. Cytochrome c Oxidase Is Required for the Assembly/Stability of Respiratory Complex I in Mouse Fibroblasts , 2006, Molecular and Cellular Biology.
[10] Matthew S. Sachs,et al. Early nonsense: mRNA decay solves a translational problem , 2006, Nature Reviews Molecular Cell Biology.
[11] E. Shoubridge,et al. A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy. , 2005, The Journal of clinical investigation.
[12] Rutger O. Vogel,et al. Human mitochondrial complex I assembly is mediated by NDUFAF1 , 2005, The FEBS journal.
[13] M. Huynen,et al. Tracing the evolution of a large protein complex in the eukaryotes, NADH:ubiquinone oxidoreductase (Complex I). , 2005, Journal of molecular biology.
[14] Joana Assunção,et al. Composition of complex I from Neurospora crassa and disruption of two "accessory" subunits. , 2005, Biochimica et biophysica acta.
[15] D. Stojanovski,et al. Dissection of the Mitochondrial Import and Assembly Pathway for Human Tom40* , 2005, Journal of Biological Chemistry.
[16] A. Ohtake,et al. Biochemical and molecular diagnosis of mitochondrial respiratory chain disorders. , 2004, Biochimica et biophysica acta.
[17] P. Bénit,et al. AIF deficiency compromises oxidative phosphorylation , 2004, The EMBO journal.
[18] Rutger O. Vogel,et al. Human mitochondrial complex I assembles through the combination of evolutionary conserved modules: a framework to interpret complex I deficiencies. , 2004, Human molecular genetics.
[19] D. Turnbull,et al. Mutations of the mitochondrial ND1 gene as a cause of MELAS , 2004, Journal of Medical Genetics.
[20] Robert W. Taylor,et al. NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency. , 2004, The Journal of clinical investigation.
[21] C. Bruno,et al. Respiratory complex III is required to maintain complex I in mammalian mitochondria. , 2004, Molecular cell.
[22] J. Smeitink,et al. Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency. , 2004, Human molecular genetics.
[23] E. Shoubridge,et al. Identification and Characterization of a Common Set of Complex I Assembly Intermediates in Mitochondria from Patients with Complex I Deficiency* , 2003, Journal of Biological Chemistry.
[24] A. Jon Stoessl,et al. Etiology of Parkinson's Disease , 2003, Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques.
[25] K. Truscott,et al. Insertion and Assembly of Human Tom7 into the Preprotein Translocase Complex of the Outer Mitochondrial Membrane* , 2002, The Journal of Biological Chemistry.
[26] R. Sprengel,et al. ER‐based double icre fusion protein allows partial recombination in forebrain , 2002, Genesis.
[27] J. Smeitink,et al. CIA30 complex I assembly factor: a candidate for human complex I deficiency? , 2002, Human Genetics.
[28] H. Schägger. Respiratory Chain Supercomplexes , 2001, IUBMB life.
[29] N. Pfanner,et al. Protein Import Channel of the Outer Mitochondrial Membrane: a Highly Stable Tom40-Tom22 Core Structure Differentially Interacts with Preproteins, Small Tom Proteins, and Import Receptors , 2001, Molecular and Cellular Biology.
[30] B. J. Hanson,et al. Human Complex I Defects Can Be Resolved by Monoclonal Antibody Analysis into Distinct Subunit Assembly Patterns* , 2001, The Journal of Biological Chemistry.
[31] K. Pfeiffer,et al. Supercomplexes in the respiratory chains of yeast and mammalian mitochondria , 2000, The EMBO journal.
[32] R. Küffner,et al. Involvement of two novel chaperones in the assembly of mitochondrial NADH:Ubiquinone oxidoreductase (complex I). , 1998, Journal of molecular biology.
[33] N Grigorieff,et al. Three-dimensional structure of bovine NADH:ubiquinone oxidoreductase (complex I) at 22 A in ice. , 1998, Journal of molecular biology.
[34] F. Gage,et al. In Vivo Gene Delivery and Stable Transduction of Nondividing Cells by a Lentiviral Vector , 1996, Science.
[35] J. Christodoulou,et al. Leigh syndrome: Clinical features and biochemical and DNA abnormalities , 1996, Annals of neurology.
[36] T. Lithgow,et al. Prechaperonin 60 and preornithine transcarbamylase share components of the import apparatus but have distinct maturation pathways in rat liver mitochondria. , 1993, European journal of biochemistry.
[37] U. Nehls,et al. Characterization of assembly intermediates of NADH:ubiquinone oxidoreductase (complex I) accumulated in Neurospora mitochondria by gene disruption. , 1992, Journal of molecular biology.
[38] H. Schägger,et al. Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form. , 1991, Analytical biochemistry.
[39] U. Nehls,et al. Assembly of NADH: ubiquinone reductase (complex I) in Neurospora mitochondria. Independent pathways of nuclear-encoded and mitochondrially encoded subunits. , 1990, Journal of molecular biology.
[40] H. Schägger,et al. Tricine-sodium dodecyl sulfate-polyacrylamide gel electrophoresis for the separation of proteins in the range from 1 to 100 kDa. , 1987, Analytical biochemistry.
[41] Marvin B. Shapiro,et al. RNA splice junctions of different classes of eukaryotes: sequence statistics and functional implications in gene expression. , 1987, Nucleic acids research.
[42] A. Dunn,et al. Effect of deletions within the leader peptide of pre-ornithine transcarbamylase on mitochondrial import. , 1986, European journal of biochemistry.
[43] Ια,et al. Energy Converting NADH : Quinone Oxidoreductase ( Complex I ) , 2012 .
[44] N. Pfanner,et al. Hsp70 proteins in protein translocation. , 2001, Advances in protein chemistry.
[45] N. Pfanner,et al. Assaying protein import into mitochondria. , 2001, Methods in cell biology.
[46] G. Lenaz,et al. Isolation and subfractionation of mitochondria from animal cells and tissue culture lines. , 2001, Methods in cell biology.
[47] E. Harlow,et al. Using Antibodies: A Laboratory Manual , 1999 .
[48] A. Chomyn. In vivo labeling and analysis of human mitochondrial translation products. , 1996, Methods in enzymology.