CERKL, a retinal disease gene, encodes an mRNA-binding protein that localizes in compact and untranslated mRNPs associated with microtubules.
CERKL, a retinal disease gene, encodes an mRNA-binding protein that localizes in compact and untranslated mRNPs associated with microtubules.
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DOI：
10.1371/journal.pone.0087898
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发表时间：
2014
期刊：
影响因子：
3.7
通讯作者：
Gonzàlez-Duarte R
中科院分区：
文献类型：
--
作者：
Fathinajafabadi A;Pérez-Jiménez E;Riera M;Knecht E;Gonzàlez-Duarte R
CERKL是视网膜色素变性和视锥视杆细胞营养不良的致病基因，其功能仍有待研究。为了探讨其在细胞中的作用，我们研究了532个氨基酸的全长CERKL亚型CERKLa在不同细胞系中的亚细胞定位和相互作用伙伴，包括光感受器来源的细胞系。我们证明CERKLa是紧密的和未翻译的mRNPs的主要成分，并与其他RNP复合体如应激颗粒、P-体和多聚体有关。CERKLa是一种通过其N端与mRNAs结合的蛋白质，并与其他mRNA结合蛋白如eIF3B、PABP、HSP70和RPS3相互作用。除了eIF3B，这些相互作用依赖于mRNAs的完整性，而不是核糖体的完整性。有趣的是，C125W CERKLA病理突变体不与eIF3B相互作用，并且不存在于这些复合体中。含有CERKLa的致密mRNPs也与微管相关，并在神经分化细胞的神经突起中发现。这些定位在视网膜疾病基因家族的任何成员以前都没有报道，当研究这些疾病的发病机制和治疗方法时，应该考虑这些定位。
The function of CERKL (CERamide Kinase Like), a causative gene of retinitis pigmentosa and cone-rod dystrophy, still awaits characterization. To approach its cellular role we have investigated the subcellular localization and interaction partners of the full length CERKL isoform, CERKLa of 532 amino acids, in different cell lines, including a photoreceptor-derived cell line. We demonstrate that CERKLa is a main component of compact and untranslated mRNPs and that associates with other RNP complexes such as stress granules, P-bodies and polysomes. CERKLa is a protein that binds through its N-terminus to mRNAs and interacts with other mRNA-binding proteins like eIF3B, PABP, HSP70 and RPS3. Except for eIF3B, these interactions depend on the integrity of mRNAs but not of ribosomes. Interestingly, the C125W CERKLa pathological mutant does not interact with eIF3B and is absent from these complexes. Compact mRNPs containing CERKLa also associate with microtubules and are found in neurites of neural differentiated cells. These localizations had not been reported previously for any member of the retinal disorders gene family and should be considered when investigating the pathogenic mechanisms and therapeutical approaches in these diseases.
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影响因子：
3.7
作者：
Karunakaran DK;Banday AR;Wu Q;Kanadia R
通讯作者：
Kanadia R
影响因子：
21.3
作者：
通讯作者：
--
影响因子：
3.7
作者：
De Leeuw, Frederic;Zhang, Tong;Gueydan, Cyril
通讯作者：
Gueydan, Cyril
DOI：
10.1016/j.bbalip.2004.11.012
发表时间：
2005-02-21
影响因子：
4.8
作者：
Bornancin, FR;Mechtcheriakova, D;Billich, A
通讯作者：
Billich, A
DOI：
10.1016/j.bbadis.2012.04.004
发表时间：
2012-08-01
影响因子：
6.2
作者：
Garanto, Alejandro;Vicente-Tejedor, Javier;Marfany, Gemma
通讯作者：
Marfany, Gemma