2007
DOI: 10.1002/jnr.21308
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QNQKE targeting motif for the SMN‐Gemin multiprotein complexin neurons

Abstract: Spinal muscular atrophy (SMA) is a heritable neurodegenerative disease affecting motor neurons that is caused by the impaired expression of the full-length form of the survival of motor neuron protein (SMN), which may have a specialized function in neurons related to mRNA localization. We have previously shown that a population SMN complexes contain Gemin ribonucleoproteins and traffic in the form of granules to neuronal processes and growth cones of cultured neurons. A QNQKE sequence within exon 7 has been sh… Show more

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Cited by 29 publications
(20 citation statements)
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“…Firstly, PABP is present in neuronal regions distant from the cell body, e.g. dendrites, axonal filopodia and terminal growth cones [62,63] and co-localizes in punta/granules with RNA-binding proteins, such as the wellcharacterized HuD [64], implicated in activity-dependent localized translation, as well as with putative regulatory factors, e.g. Makorin RING zinc finger protein (MKNR)1 [65], with which it interacts.…”
Section: Pabp and Localized Translationmentioning
confidence: 99%
“…Firstly, PABP is present in neuronal regions distant from the cell body, e.g. dendrites, axonal filopodia and terminal growth cones [62,63] and co-localizes in punta/granules with RNA-binding proteins, such as the wellcharacterized HuD [64], implicated in activity-dependent localized translation, as well as with putative regulatory factors, e.g. Makorin RING zinc finger protein (MKNR)1 [65], with which it interacts.…”
Section: Pabp and Localized Translationmentioning
confidence: 99%
“…These data indicate that the number or structural integrity of gems may not be directly relevant to SMA disease progression. Instead, other nuclear or axonal SMN functions could be responsible for motor neuron degeneration (38)(39)(40)(41)(42)(43)(44). Furthermore, only a subset of SMN molecules is bound by FGF-2 23 (21,22).…”
Section: Discussionmentioning
confidence: 99%
“…The RFP-hSMN (Zhang et al, 2007) in pCS2 was digested with Bam HI and Not I and cloned into pBluescript vector that contained 2 SceI sites (RFP-hSMN/pBluescripSceI plasmid). The 1.5 kb heat shock 70 promoter ( hsp70 ) was released from the pHSP70/4 EGFP-1 plasmid (Halloran et al, 2000) using the Bam HI site and cloned into the RFP-hSMN/pBluescript plasmid containing I-SceI sites to enhance transgenesis (Thermes et al, 2002).…”
Section: Methodsmentioning
confidence: 99%