In vivo mutation of pre-mRNA processing factor 8 (Prpf8) affects transcript splicing, cell survival and myeloid differentiation

Keightley, Maria-Cristina, Crowhurst, Meredith O., Layton, Judith E., Beilharz, Traude, Markmiller, Sebastian, Varma, Sony, Hogan, Benjamin M., de Jong-Curtain, Tanya A., Heath, Joan K. and Lieschke, Graham J. (2013) In vivo mutation of pre-mRNA processing factor 8 (Prpf8) affects transcript splicing, cell survival and myeloid differentiation. Febs Letters, 587 14: 2150-2157. doi:10.1016/j.febslet.2013.05.030


Author Keightley, Maria-Cristina
Crowhurst, Meredith O.
Layton, Judith E.
Beilharz, Traude
Markmiller, Sebastian
Varma, Sony
Hogan, Benjamin M.
de Jong-Curtain, Tanya A.
Heath, Joan K.
Lieschke, Graham J.
Title In vivo mutation of pre-mRNA processing factor 8 (Prpf8) affects transcript splicing, cell survival and myeloid differentiation
Journal name Febs Letters   Check publisher's open access policy
ISSN 0014-5793
1873-3468
Publication date 2013-07-01
Year available 2013
Sub-type Article (original research)
DOI 10.1016/j.febslet.2013.05.030
Open Access Status DOI
Volume 587
Issue 14
Start page 2150
End page 2157
Total pages 8
Place of publication Amsterdam, The Netherlands
Publisher Elsevier BV
Language eng
Formatted abstract
Mutated spliceosome components are recurrently being associated with perturbed tissue development and disease pathogenesis. CephalophoÌ †nus (cph), is a zebrafish mutant carrying an early premature STOP codon in the spliceosome component Prpf8 (pre-mRNA processing factor 8). Cph initially develops normally, but then develops widespread cell death, especially in neurons, and is embryonic lethal. Cph mutants accumulate aberrantly spliced transcripts retaining both U2- and U12-type introns. Within early haematopoiesis, myeloid differentiation is impaired, suggesting Prpf8 is required for haematopoietic development. Cph provides an animal model for zygotic PRPF8 dysfunction diseases and for evaluating therapeutic interventions.
Keyword Haematopoiesis
Splicing
Zebrafish
Myelopoiesis
Spliceosome
Q-Index Code C1
Q-Index Status Provisional Code
Grant ID RO1 HL079545
234708
DP0346823
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
Institute for Molecular Bioscience - Publications
 
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Citation counts: TR Web of Science Citation Count  Cited 17 times in Thomson Reuters Web of Science Article | Citations
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Created: Wed, 11 Dec 2013, 01:50:55 EST by Susan Allen on behalf of Institute for Molecular Bioscience