Regulation of a minimal transcriptome by repeat domain proteins

Research output: Contribution to journalReview article

4 Citations (Scopus)
76 Downloads (Pure)

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 languageEnglish
Pages (from-to)132-141
JournalSeminars in Cell and Developmental Biology
Volume76
DOIs
Publication statusPublished - Apr 2018

Fingerprint

Mitochondrial Genes
Transcriptome
RNA Sequence Analysis
Mitochondrial Diseases
Mitochondrial Genome
Mitochondrial Proteins
Genetic Models
Gene Expression Regulation
Proteins
Technology
Gene Expression
Mutation
Genes
Protein Domains

Cite this

@article{373e95692ec14e78a3b625c638b4869b,
title = "Regulation of a minimal transcriptome by repeat domain proteins",
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.",
keywords = "Gene expression, Mitochondrial RNA, Mitochondrial transcription termination factor, Pentatricopeptide repeat protein, RNA-binding protein",
author = "Nicola Ferreira and Oliver Rackham and Aleksandra Filipovska",
year = "2018",
month = "4",
doi = "10.1016/j.semcdb.2017.08.037",
language = "English",
volume = "76",
pages = "132--141",
journal = "Seminars in Cell & Developmental Biology",
issn = "1084-9521",
publisher = "Academic Press",

}

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 journalReview 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 -