Concepedia

Mitochondrial Rna Processing Defects

Mitochondrial RNA Processing Defects is a molecular pathogenic condition characterized by the disruption of post-transcriptional modifications, splicing, cleavage, or maturation of mitochondrial RNA transcripts, leading to impaired mitochondrial gene expression and respiratory chain dysfunction.

185

Publications

11.3K

Citations

1.1K

Authors

408

Institutions

Publications per year

2017–2026

34

Parents

Publications

185

Maternally Inherited Aminoglycoside-Induced and Nonsyndromic Deafness Is Associated with the Novel C1494T Mutation in the Mitochondrial 12S rRNA Gene in a Large Chinese Family

Hui Zhao, Ronghua Li, Qiuju Wang et al. · The American Journal of Human Genetics · 2004 · 424 citations · Full text

, , +17

Codon-specific translational defect caused by a wobble modification deficiency in mutant tRNA from a human mitochondrial disease

Yohei Kirino, Takehiro Yasukawa, Shigeo Ohta et al. · Proceedings of the National Academy of Sciences · 2004 · 288 citations · Full text

, , +17

Modification Defect at Anticodon Wobble Nucleotide of Mitochondrial tRNAsLeu(UUR) with Pathogenic Mutations of Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-like Episodes

Takehiro Yasukawa, Tsutomu Suzuki, Takeo Suzuki et al. · Journal of Biological Chemistry · 2000 · 269 citations · Full text

, , +17

A pentatricopeptide repeat‐containing gene that promotes the processing of aberrant <i>atp6</i> RNA of cytoplasmic male‐sterile rice

Tomohiko Kazama, Kinya Toriyama · FEBS Letters · 2003 · 261 citations · Full text

, , +14

Wobble modification defect in tRNA disturbs codon–anticodon interaction in a mitochondrial disease

Takehiro Yasukawa, Tsutomu Suzuki, Norie Ishii et al. · The EMBO Journal · 2001 · 237 citations · Full text

, , +15

1–5 of 185

Authors

1.1K

  1. MG
    Min‐Xin Guan

    Cincinnati Children's Hospital Medical Center, United States

    19 publications · 1.7K citations

    19

    h-index

  2. TS
    Tsutomu Suzuki

    Hoshi University, Japan

    9 publications · 1.6K citations

    9

    h-index

  3. RJ
    Rudolf J. Schweyen

    University of Vienna, Austria

    6 publications · 408 citations

    6

    h-index

  4. AM
    Alan M. Lambowitz

    The University of Texas at Austin, United States

    6 publications · 311 citations

    6

    h-index

  5. PP
    Piotr P. Słonimski

    Centre de Génétique Moléculaire, France

    5 publications · 244 citations

    5

    h-index

1–5 of 1.1K

Institutions

408

  1. Cincinnati Children's Hospital Medical Center

    Cincinnati, United States

    12 h-index

    43 publications

    5.7K citations

  2. Centre National de la Recherche Scientifique

    Paris, France

    12 h-index

    44 publications

    2.7K citations

  3. University of Cincinnati Medical Center

    Cincinnati, United States

    11 h-index

    12 publications

    1K citations

  4. The University of Tokyo

    Tokyo, Japan

    10 h-index

    48 publications

    8.3K citations

  5. Zhejiang University

    Hangzhou, China

    8 h-index

    52 publications

    2.1K citations

  6. Wenzhou Medical University

    Wenzhou, China

    7 h-index

    47 publications

    5.3K citations

  7. Centre de Génétique Moléculaire

    Gif-sur-Yvette, France

    7 h-index

    27 publications

    1K citations

  8. Columbia University

    New York, United States

    6 h-index

    23 publications

    2.2K citations

1–8 of 408

Venues

  1. Journal of Biological Chemistry

    JBC · 19 publications · 1.6K citations

    18

    h-index

  2. Nucleic Acids Research

    18 publications · 891 citations

    17

    h-index

  3. Biochemical and Biophysical Research Communications

    BBRC · 9 publications · 333 citations

    9

    h-index

  4. Mitochondrion

    8 publications · 344 citations

    8

    h-index

  5. Human Molecular Genetics

    7 publications · 456 citations

    7

    h-index

1–5