Abstract
| Original language | English |
|---|---|
| Pages (from-to) | 2150 - 2157 |
| Number of pages | 8 |
| Journal | FEBS Letters |
| Volume | 587 |
| Issue number | 14 |
| DOIs | |
| Publication status | Published - 2013 |
Cite this
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In vivo mutation of pre-mRNA processing factor 8 (Prpf8) affects transcript splicing, cell survival and myeloid differentiation. / Keightley, Maria-Cristina; Crowhurst, Meredith; Layton, Judith E; Beilharz, Traude Helene; Markmiller, Sebastian J; Varma, Sony; Hogan, Benjamin M; de Jong-Curtain, Tanya A; Heath, Joan K; Lieschke, Graham.
In: FEBS Letters, Vol. 587, No. 14, 2013, p. 2150 - 2157.Research output: Contribution to journal › Article › Research › peer-review
TY - JOUR
T1 - In vivo mutation of pre-mRNA processing factor 8 (Prpf8) affects transcript splicing, cell survival and myeloid differentiation
AU - Keightley, Maria-Cristina
AU - Crowhurst, Meredith
AU - Layton, Judith E
AU - Beilharz, Traude Helene
AU - Markmiller, Sebastian J
AU - Varma, Sony
AU - Hogan, Benjamin M
AU - de Jong-Curtain, Tanya A
AU - Heath, Joan K
AU - Lieschke, Graham
PY - 2013
Y1 - 2013
N2 - Mutated spliceosome components are recurrently being associated with perturbed tissue development and disease pathogenesis. Cephalophonus (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.
AB - Mutated spliceosome components are recurrently being associated with perturbed tissue development and disease pathogenesis. Cephalophonus (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.
UR - http://www.ncbi.nlm.nih.gov/pubmed/23714367
U2 - 10.1016/j.febslet.2013.05.030
DO - 10.1016/j.febslet.2013.05.030
M3 - Article
VL - 587
SP - 2150
EP - 2157
JO - FEBS Letters
JF - FEBS Letters
SN - 0014-5793
IS - 14
ER -