MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.

Christin Tischner; Annette Hofer; Veronika Wulff; Joanna Stepek; Iulia Dumitru; Lore Becker; Tobias Haack; Laura Kremer; Alexandre N Datta; Wolfgang Sperl; Thomas Floss; Wolfgang Wurst; Zofia Chrzanowska-Lightowlers; Martin Hrabe De Angelis; Thomas Klopstock; Holger Prokisch; Tina Wenz

doi:10.1093/hmg/ddu743

MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.

Christin Tischner
, Annette Hofer
, Veronika Wulff
, Joanna Stepek
, Iulia Dumitru
, Lore Becker
, Tobias Haack
, Laura Kremer
, Alexandre N Datta
, Wolfgang Sperl (Co-author)
, Thomas Floss
, Wolfgang Wurst
, Zofia Chrzanowska-Lightowlers
, Martin Hrabe De Angelis
, Thomas Klopstock
, Holger Prokisch
, Tina Wenz

Department of Pediatrics

Research output: Contribution to journal › Original Article › peer-review

42 Citations (Web of Science)

Original language	English
Pages (from-to)	2247-2266
Journal	HUMAN MOLECULAR GENETICS
Volume	24
Issue number	8
DOIs	https://doi.org/10.1093/hmg/ddu743
Publication status	Published - 2015

Keywords

WOBBLE MODIFICATION DEFICIENCY
RESPIRATORY-CHAIN DEFICIENCY
HUMAN MITOCHONDRIAL DISEASES
MUTANT TRANSFER-RNAS
OXIDATIVE-PHOSPHORYLATION
LACTIC-ACIDOSIS
IN-VIVO
HYPERTROPHIC CARDIOMYOPATHY
PROTEIN-SYNTHESIS
ESCHERICHIA-COLI

Access to Document

10.1093/hmg/ddu743

Cite this

Tischner, C., Hofer, A., Wulff, V., Stepek, J., Dumitru, I., Becker, L., Haack, T., Kremer, L., Datta, A. N., Sperl, W., Floss, T., Wurst, W., Chrzanowska-Lightowlers, Z., De Angelis, M. H., Klopstock, T., Prokisch, H., & Wenz, T. (2015). MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention. HUMAN MOLECULAR GENETICS , 24(8), 2247-2266. https://doi.org/10.1093/hmg/ddu743

Tischner, Christin ; Hofer, Annette ; Wulff, Veronika et al. / MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention. In: HUMAN MOLECULAR GENETICS 2015 ; Vol. 24, No. 8. pp. 2247-2266.

@article{7edb026840654c728924025d6aa3c40b,

title = "MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.",

keywords = "WOBBLE MODIFICATION DEFICIENCY, RESPIRATORY-CHAIN DEFICIENCY, HUMAN MITOCHONDRIAL DISEASES, MUTANT TRANSFER-RNAS, OXIDATIVE-PHOSPHORYLATION, LACTIC-ACIDOSIS, IN-VIVO, HYPERTROPHIC CARDIOMYOPATHY, PROTEIN-SYNTHESIS, ESCHERICHIA-COLI",

author = "Christin Tischner and Annette Hofer and Veronika Wulff and Joanna Stepek and Iulia Dumitru and Lore Becker and Tobias Haack and Laura Kremer and Datta, \{Alexandre N\} and Wolfgang Sperl and Thomas Floss and Wolfgang Wurst and Zofia Chrzanowska-Lightowlers and \{De Angelis\}, \{Martin Hrabe\} and Thomas Klopstock and Holger Prokisch and Tina Wenz",

note = "8Department of Pediatrics, Paracelsus Medical University Salzburg, Salzburg, Austria, ",

year = "2015",

doi = "10.1093/hmg/ddu743",

language = "English",

volume = "24",

pages = "2247--2266",

journal = "HUMAN MOLECULAR GENETICS ",

issn = "0964-6906",

number = "8",

}

Tischner, C, Hofer, A, Wulff, V, Stepek, J, Dumitru, I, Becker, L, Haack, T, Kremer, L, Datta, AN, Sperl, W, Floss, T, Wurst, W, Chrzanowska-Lightowlers, Z, De Angelis, MH, Klopstock, T, Prokisch, H & Wenz, T 2015, 'MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.', HUMAN MOLECULAR GENETICS , vol. 24, no. 8, pp. 2247-2266. https://doi.org/10.1093/hmg/ddu743

MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention. / Tischner, Christin; Hofer, Annette; Wulff, Veronika et al.
In: HUMAN MOLECULAR GENETICS , Vol. 24, No. 8, 2015, p. 2247-2266.

Research output: Contribution to journal › Original Article › peer-review

TY - JOUR

T1 - MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention.

AU - Tischner, Christin

AU - Hofer, Annette

AU - Wulff, Veronika

AU - Stepek, Joanna

AU - Dumitru, Iulia

AU - Becker, Lore

AU - Haack, Tobias

AU - Kremer, Laura

AU - Datta, Alexandre N

AU - Sperl, Wolfgang

AU - Floss, Thomas

AU - Wurst, Wolfgang

AU - Chrzanowska-Lightowlers, Zofia

AU - De Angelis, Martin Hrabe

AU - Klopstock, Thomas

AU - Prokisch, Holger

AU - Wenz, Tina

N1 - 8Department of Pediatrics, Paracelsus Medical University Salzburg, Salzburg, Austria,

PY - 2015

Y1 - 2015

KW - WOBBLE MODIFICATION DEFICIENCY

KW - RESPIRATORY-CHAIN DEFICIENCY

KW - HUMAN MITOCHONDRIAL DISEASES

KW - MUTANT TRANSFER-RNAS

KW - OXIDATIVE-PHOSPHORYLATION

KW - LACTIC-ACIDOSIS

KW - IN-VIVO

KW - HYPERTROPHIC CARDIOMYOPATHY

KW - PROTEIN-SYNTHESIS

KW - ESCHERICHIA-COLI

U2 - 10.1093/hmg/ddu743

DO - 10.1093/hmg/ddu743

M3 - Original Article

C2 - 25552653

SN - 0964-6906

VL - 24

SP - 2247

EP - 2266

JO - HUMAN MOLECULAR GENETICS

JF - HUMAN MOLECULAR GENETICS

IS - 8

ER -

Tischner C, Hofer A, Wulff V, Stepek J, Dumitru I, Becker L et al. MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention. HUMAN MOLECULAR GENETICS . 2015;24(8):2247-2266. doi: 10.1093/hmg/ddu743