Abstract
Repeat proteins regulate the expression of the mammalian mitochondrial genome at the level of transcription, processing, maturation, and translation. Defects in the regulation of mitochondrial gene expression due to mutations in genes encoding repeat proteins can lead to mitochondrial dysfunction and disease, however the molecular mechanisms that regulate mitochondrial gene expression and how defects in these processes cause disease still remains poorly understood. Recently solved crystal structures, characterisation of the new genetic models, and use of RNA sequencing (RNA-Seq) technologies have greatly expanded our current understanding of mitochondrial repeat proteins and biology.
| Original language | English |
|---|---|
| Pages (from-to) | 132-141 |
| Journal | Seminars in Cell and Developmental Biology |
| Volume | 76 |
| DOIs | |
| Publication status | Published - Apr 2018 |
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Regulation of a minimal transcriptome by repeat domain proteins. / Ferreira, Nicola; Rackham, Oliver; Filipovska, Aleksandra.
In: Seminars in Cell and Developmental Biology, Vol. 76, 04.2018, p. 132-141.Research output: Contribution to journal › Review article
TY - JOUR
T1 - Regulation of a minimal transcriptome by repeat domain proteins
AU - Ferreira, Nicola
AU - Rackham, Oliver
AU - Filipovska, Aleksandra
PY - 2018/4
Y1 - 2018/4
N2 - Repeat proteins regulate the expression of the mammalian mitochondrial genome at the level of transcription, processing, maturation, and translation. Defects in the regulation of mitochondrial gene expression due to mutations in genes encoding repeat proteins can lead to mitochondrial dysfunction and disease, however the molecular mechanisms that regulate mitochondrial gene expression and how defects in these processes cause disease still remains poorly understood. Recently solved crystal structures, characterisation of the new genetic models, and use of RNA sequencing (RNA-Seq) technologies have greatly expanded our current understanding of mitochondrial repeat proteins and biology.
AB - Repeat proteins regulate the expression of the mammalian mitochondrial genome at the level of transcription, processing, maturation, and translation. Defects in the regulation of mitochondrial gene expression due to mutations in genes encoding repeat proteins can lead to mitochondrial dysfunction and disease, however the molecular mechanisms that regulate mitochondrial gene expression and how defects in these processes cause disease still remains poorly understood. Recently solved crystal structures, characterisation of the new genetic models, and use of RNA sequencing (RNA-Seq) technologies have greatly expanded our current understanding of mitochondrial repeat proteins and biology.
KW - Gene expression
KW - Mitochondrial RNA
KW - Mitochondrial transcription termination factor
KW - Pentatricopeptide repeat protein
KW - RNA-binding protein
UR - http://www.scopus.com/inward/record.url?scp=85029653036&partnerID=8YFLogxK
U2 - 10.1016/j.semcdb.2017.08.037
DO - 10.1016/j.semcdb.2017.08.037
M3 - Review article
VL - 76
SP - 132
EP - 141
JO - Seminars in Cell & Developmental Biology
JF - Seminars in Cell & Developmental Biology
SN - 1084-9521
ER -