Aberrant 3′ oligoadenylation of spliceosomal U6 small nuclear RNA in poikiloderma with neutropenia-Reference-Cited by-同舟云学术

Aberrant 3′ oligoadenylation of spliceosomal U6 small nuclear RNA in poikiloderma with neutropenia

Published:2013-02-07 Issue:6 Volume:121 Page:1028-1038
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Hilcenko Christine¹²³,Simpson Paul J.¹²³,Finch Andrew J.¹²³,Bowler Frank R.¹,Churcher Mark J.¹²³,Jin Li¹²³,Packman Len C.⁴,Shlien Adam⁵,Campbell Peter²⁵,Kirwan Michael⁶,Dokal Inderjeet⁶,Warren Alan J.¹²³

Affiliation:

1. MRC Laboratory of Molecular Biology, Cambridge, United Kingdom;

2. The Department of Haematology, University of Cambridge, Cambridge, United Kingdom;

3. Wellcome Trust–Medical Research Council Stem Cell Institute, University of Cambridge, Cambridge, United Kingdom;

4. Department of Biochemistry, University of Cambridge, Cambridge, United Kingdom;

5. Cancer Genome Project, Wellcome Trust Sanger Institute, Hinxton, United Kingdom; and

6. Centre for Paediatrics, Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom

Abstract

Key PointsCrystal structure of human USB1 identifies it as a member of the LigT-like superfamily of 2H phosphoesterases. USB1 protects spliceosomal U6 small nuclear RNA from aberrant 3′ oligoadenylation.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/121/6/1028/1367726/zh800613001028.pdf

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