Multiple interaction partners for Cockayne syndrome proteins: Implications for genome and transcriptome maintenance

Maria D Aamann; Meltem Muftuoglu; Vilhelm A Bohr; Tinna Stevnsner

doi:10.1016/j.mad.2013.03.009

Multiple interaction partners for Cockayne syndrome proteins: Implications for genome and transcriptome maintenance

Maria D Aamann, Meltem Muftuoglu, Vilhelm A Bohr, Tinna Stevnsner

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Review › Research › peer-review

30 Citations (Scopus)

Abstract

Cockayne syndrome (CS) is characterized by progressive multisystem degeneration and is classified as a segmental premature aging syndrome. The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein. Cells from CS patients are sensitive to UV light and a number of other DNA damaging agents including various types of oxidative stress. The cells also display transcription deficiencies, abnormal apoptotic response to DNA damage, and DNA repair deficiencies. Herein we have critically reviewed the current knowledge about known protein interactions of the CS proteins. The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.

Original language	English
Journal	Mechanisms of Ageing and Development
Volume	134
Issue	5-6
Pages (from-to)	212-224
Number of pages	13
ISSN	0047-6374
DOIs	https://doi.org/10.1016/j.mad.2013.03.009
Publication status	Published - May 2013

Keywords

Animals
DNA Damage
DNA Helicases
DNA Repair
DNA Repair Enzymes
Genome, Human
Humans
Transcription Factors
Transcriptome

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10.1016/j.mad.2013.03.009

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title = "Multiple interaction partners for Cockayne syndrome proteins: Implications for genome and transcriptome maintenance",

abstract = "Cockayne syndrome (CS) is characterized by progressive multisystem degeneration and is classified as a segmental premature aging syndrome. The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein. Cells from CS patients are sensitive to UV light and a number of other DNA damaging agents including various types of oxidative stress. The cells also display transcription deficiencies, abnormal apoptotic response to DNA damage, and DNA repair deficiencies. Herein we have critically reviewed the current knowledge about known protein interactions of the CS proteins. The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.",

keywords = "Animals, DNA Damage, DNA Helicases, DNA Repair, DNA Repair Enzymes, Genome, Human, Humans, Transcription Factors, Transcriptome",

author = "Aamann, {Maria D} and Meltem Muftuoglu and Bohr, {Vilhelm A} and Tinna Stevnsner",

year = "2013",

month = may,

doi = "10.1016/j.mad.2013.03.009",

language = "English",

volume = "134",

pages = "212--224",

journal = "Mechanisms of Ageing and Development",

issn = "0047-6374",

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Multiple interaction partners for Cockayne syndrome proteins: Implications for genome and transcriptome maintenance. / Aamann, Maria D; Muftuoglu, Meltem; Bohr, Vilhelm A et al.
In: Mechanisms of Ageing and Development, Vol. 134, No. 5-6, 05.2013, p. 212-224.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Review › Research › peer-review

TY - JOUR

T1 - Multiple interaction partners for Cockayne syndrome proteins

T2 - Implications for genome and transcriptome maintenance

AU - Aamann, Maria D

AU - Muftuoglu, Meltem

AU - Bohr, Vilhelm A

AU - Stevnsner, Tinna

PY - 2013/5

Y1 - 2013/5

N2 - Cockayne syndrome (CS) is characterized by progressive multisystem degeneration and is classified as a segmental premature aging syndrome. The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein. Cells from CS patients are sensitive to UV light and a number of other DNA damaging agents including various types of oxidative stress. The cells also display transcription deficiencies, abnormal apoptotic response to DNA damage, and DNA repair deficiencies. Herein we have critically reviewed the current knowledge about known protein interactions of the CS proteins. The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.

AB - Cockayne syndrome (CS) is characterized by progressive multisystem degeneration and is classified as a segmental premature aging syndrome. The majority of CS cases are caused by defects in the CS complementation group B (CSB) protein and the rest are mainly caused by defects in the CS complementation group A (CSA) protein. Cells from CS patients are sensitive to UV light and a number of other DNA damaging agents including various types of oxidative stress. The cells also display transcription deficiencies, abnormal apoptotic response to DNA damage, and DNA repair deficiencies. Herein we have critically reviewed the current knowledge about known protein interactions of the CS proteins. The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.

KW - Animals

KW - DNA Damage

KW - DNA Helicases

KW - DNA Repair

KW - DNA Repair Enzymes

KW - Genome, Human

KW - Humans

KW - Transcription Factors

KW - Transcriptome

U2 - 10.1016/j.mad.2013.03.009

DO - 10.1016/j.mad.2013.03.009

M3 - Review

C2 - 23583689

SN - 0047-6374

VL - 134

SP - 212

EP - 224

JO - Mechanisms of Ageing and Development

JF - Mechanisms of Ageing and Development

IS - 5-6

ER -

Multiple interaction partners for Cockayne syndrome proteins: Implications for genome and transcriptome maintenance

Abstract

Keywords

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