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{{short description|Protein in animal cells}}
{{Infobox protein family
{{Infobox protein family
| Symbol = SMN
| Symbol = SMN
| Name = Survival motor neuron protein
| Name = Survival motor neuron protein
| image = Protein_SMN1_PDB_1g5v.png
| image = Protein_SMN1_PDB_1g5v.png
| width =
| width =
| caption = Tudor domain from human SMN. PDB {{PDBe|1g5v}}<ref>{{cite journal | vauthors = Selenko P, Sprangers R, Stier G, Bühler D, Fischer U, Sattler M | title = SMN tudor domain structure and its interaction with the Sm proteins | journal = Nature Structural Biology | volume = 8 | issue = 1 | pages = 27–31 | date = January 2001 | pmid = 11135666 | pmc = | doi = 10.1038/83014 }}</ref>
| caption = Tudor domain from human SMN. PDB {{PDBe|1g5v}}<ref>{{cite journal | vauthors = Selenko P, Sprangers R, Stier G, Bühler D, Fischer U, Sattler M | title = SMN tudor domain structure and its interaction with the Sm proteins | journal = Nature Structural Biology | volume = 8 | issue = 1 | pages = 27–31 | date = January 2001 | pmid = 11135666 | doi = 10.1038/83014 }}</ref>
| Pfam = PF06003
| Pfam = PF06003
| Pfam_clan = CL0049
| Pfam_clan = CL0049
| InterPro = IPR010304
| InterPro = IPR010304
| SMART =
| SMART =
| PROSITE =
| PROSITE =
| MEROPS =
| MEROPS =
| SCOP = 1mhn
| SCOP = 1mhn
| TCDB =
| TCDB =
| OPM family =
| OPM family =
| OPM protein =
| OPM protein =
| CAZy =
| CAZy =
| CDD =
| CDD =
}}
}}
'''Survival of motor neuron''' ('''SMN''') is a [[protein]] that in humans is encoded by the ''[[SMN1]]'' and ''[[SMN2]]'' [[gene]]s.
'''Survival of motor neuron''' or '''survival motor neuron''' ('''SMN''') is a [[protein]] that in humans is encoded by the ''[[SMN1]]'' and ''[[SMN2]]'' [[gene]]s.


⚫
SMN is found in the cytoplasm of all animal cells and also in the [[cell nucleus|nuclear]] [[cell nucleus#Cajal bodies and gems|gems]]. It functions in [[transcriptional regulation]], [[telomerase]] regeneration and [[active transport|cellular trafficking]].<ref>{{cite journal | vauthors = Singh NN, Shishimorova M, Cao LC, Gangwani L, Singh RN | title = A short antisense oligonucleotide masking a unique intronic motif prevents skipping of a critical exon in spinal muscular atrophy | journal = RNA Biology | volume = 6 | issue = 3 | pages = 341–50 | year = 2009 | pmid = 19430205 | pmc = 2734876 | doi=10.4161/rna.6.3.8723}}</ref> SMN deficiency, primarily due to [[mutation]]s in ''SMN1'', results in widespread [[RNA splicing|splicing]] defects, especially in spinal [[motor neuron]]s, and is one cause of [[spinal muscular atrophy]]. Research also showed a possible role of SMN in [[neuronal migration]] and/or [[cellular differentiation|differentiation]].<ref>{{cite journal | vauthors = Giavazzi A, Setola V, Simonati A, Battaglia G | title = Neuronal-specific roles of the survival motor neuron protein: evidence from survival motor neuron expression patterns in the developing human central nervous system | journal = Journal of Neuropathology and Experimental Neurology | volume = 65 | issue = 3 | pages = 267–77 | date = March 2006 | pmid = 16651888 | doi = 10.1097/01.jnen.0000205144.54457.a3 | doi-access = free }}</ref>
⚫
Sometimes the name is also used to refer to the entire multi-protein complex involved in the [[assembly]] of [[snRNP]]s, the essential components of [[spliceosome|spliceosomal machinery]].<ref name="Gubitz">{{cite journal | vauthors = Gubitz AK, Feng W, Dreyfuss G | title = The SMN complex | journal = Experimental Cell Research | volume = 296 | issue = 1 | pages = 51–6 | date = May 2004 | pmid = 15120993 | doi = 10.1016/j.yexcr.2004.03.022 }}</ref> The complex, apart from the survival of motor neuron protein, includes at least six other proteins ([[gem-associated protein]]s 2–7) <ref name =Gubitz />


== Function ==
⚫
SMN is found in the cytoplasm of all animal cells and also in the nuclear ''gems''. It functions in [[transcriptional regulation]], [[telomerase]] regeneration and [[active transport|cellular trafficking]].<ref>{{Cite journal|vauthors=Singh NN, Shishimorova M, Cao LC, Gangwani L, Singh RN |title=A short antisense oligonucleotide masking a unique intronic motif prevents skipping of a critical exon in spinal muscular atrophy |journal=RNA Biology |volume=6 |issue=3 |pages=341–50 |year=2009 |pmid=19430205 |pmc=2734876 }}</ref> SMN deficiency, primarily due to [[mutation]]s in ''SMN1'', results in widespread [[splicing]] defects, especially in spinal [[motor neuron]]s, and is one cause of [[spinal muscular atrophy]]. Research also showed a possible role of SMN in [[neuronal migration]] and/or [[cellular differentiation|differentiation]].<ref>{{cite journal |pmid=16651888|year=2006|author1=Giavazzi|first1=A|title=Neuronal-specific roles of the survival motor neuron protein: Evidence from survival motor neuron expression patterns in the developing human central nervous system|journal=Journal of Neuropathology and Experimental Neurology|volume=65|issue=3|pages=267–77|last2=Setola|first2=V|last3=Simonati|first3=A|last4=Battaglia|first4=G|doi=10.1097/01.jnen.0000205144.54457.a3}}</ref>
The SMN protein contains [[GEMIN2 (gene)|GEMIN2]]-binding, [[Tudor domain|Tudor]] and YG-Box domains.<ref name=pmcid3519385>{{cite journal | vauthors = Martin R, Gupta K, Ninan NS, Perry K, Van Duyne GD | title = The survival motor neuron protein forms soluble glycine zipper oligomers | journal = Structure | volume = 20 | issue = 11 | pages = 1929–39 | date = November 2012 | pmid = 23022347 | pmc = 3519385 | doi = 10.1016/j.str.2012.08.024 }}</ref> It localizes to both the [[cytoplasm]] and the [[Cell nucleus|nucleus]]. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small [[ribonucleoprotein]]s (snRNPs). This protein forms heteromeric complexes with proteins such as GEMIN2 and [[GEMIN4]], and also interacts with several proteins known to be involved in the [[biogenesis]] of [[snRNP]]s, such as hnRNP U protein and the small nucleolar RNA binding protein.<ref name="entrez">{{cite web | title = Entrez Gene: SMN1 survival of motor neuron 1, telomeric| url = https://www.ncbi.nlm.nih.gov/gene/6606}}</ref>

== SMN complex ==
⚫
''SMN complex'' refers to the entire multi-protein complex involved in the assembly of [[snRNP]]s, the essential components of [[spliceosome|spliceosomal machinery]].<ref name="Gubitz">{{cite journal | vauthors = Gubitz AK, Feng W, Dreyfuss G | title = The SMN complex | journal = Experimental Cell Research | volume = 296 | issue = 1 | pages = 51–6 | date = May 2004 | pmid = 15120993 | doi = 10.1016/j.yexcr.2004.03.022 }}</ref> The complex, apart from the "proper" survival of motor neuron protein, includes at least six other proteins ([[gem-associated protein 2]], [[DDX20|3]], [[gem-associated protein 4|4]], [[gem-associated protein 5|5]], [[gem-associated protein 6|6]] and [[gem-associated protein 7|7]].<ref name =Gubitz />

== Interactions ==

SMN has been shown to [[Protein-protein interaction|interact]] with:
{{div col|colwidth=20em}}
* [[Bcl-2]],<ref name=pmid9389483>{{cite journal | vauthors = Iwahashi H, Eguchi Y, Yasuhara N, Hanafusa T, Matsuzawa Y, Tsujimoto Y | title = Synergistic anti-apoptotic activity between Bcl-2 and SMN implicated in spinal muscular atrophy | journal = Nature | volume = 390 | issue = 6658 | pages = 413–7 | date = November 1997 | pmid = 9389483 | doi = 10.1038/37144 | bibcode = 1997Natur.390..413I }}</ref>
* [[Coilin]],<ref name=pmid12361597>{{cite journal | vauthors = Hebert MD, Shpargel KB, Ospina JK, Tucker KE, Matera AG | title = Coilin methylation regulates nuclear body formation | journal = Developmental Cell | volume = 3 | issue = 3 | pages = 329–37 | date = September 2002 | pmid = 12361597 | doi = 10.1016/S1534-5807(02)00222-8 | doi-access = free }}</ref><ref name=pmid11641277>{{cite journal | vauthors = Hebert MD, Szymczyk PW, Shpargel KB, Matera AG | title = Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein | journal = Genes & Development | volume = 15 | issue = 20 | pages = 2720–9 | date = October 2001 | pmid = 11641277 | pmc = 312817 | doi = 10.1101/gad.908401 }}</ref>
* [[DDX20]],<ref name=pmid10942426/><ref name=pmid11914277>{{cite journal | vauthors = Mourelatos Z, Dostie J, Paushkin S, Sharma A, Charroux B, Abel L, [[Juri Rappsilber|Rappsilber J]], Mann M, Dreyfuss G | title = miRNPs: a novel class of ribonucleoproteins containing numerous microRNAs | journal = Genes & Development | volume = 16 | issue = 6 | pages = 720–8 | date = March 2002 | pmid = 11914277 | pmc = 155365 | doi = 10.1101/gad.974702 }}</ref><ref name=pmid10601333>{{cite journal | vauthors = Charroux B, Pellizzoni L, Perkinson RA, Shevchenko A, Mann M, Dreyfuss G | title = Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems | journal = The Journal of Cell Biology | volume = 147 | issue = 6 | pages = 1181–94 | date = December 1999 | pmid = 10601333 | pmc = 2168095 | doi = 10.1083/jcb.147.6.1181 }}</ref>
* [[DHX9]],<ref name=pmid11149922>{{cite journal | vauthors = Pellizzoni L, Charroux B, Rappsilber J, Mann M, Dreyfuss G | title = A functional interaction between the survival motor neuron complex and RNA polymerase II | journal = The Journal of Cell Biology | volume = 152 | issue = 1 | pages = 75–85 | date = January 2001 | pmid = 11149922 | pmc = 2193649 | doi = 10.1083/jcb.152.1.75 }}</ref>
* [[Fibrillarin|FBL]],<ref name=pmid11509230>{{cite journal | vauthors = Pellizzoni L, Baccon J, Charroux B, Dreyfuss G | title = The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1 | journal = Current Biology | volume = 11 | issue = 14 | pages = 1079–88 | date = July 2001 | pmid = 11509230 | doi = 10.1016/S0960-9822(01)00316-5 | doi-access = free | bibcode = 2001CBio...11.1079P }}</ref>
* [[Far upstream element-binding protein 1|FUBP1]],<ref name=pmid10734235>{{cite journal | vauthors = Williams BY, Hamilton SL, Sarkar HK | title = The survival motor neuron protein interacts with the transactivator FUSE binding protein from human fetal brain | journal = FEBS Letters | volume = 470 | issue = 2 | pages = 207–10 | date = March 2000 | pmid = 10734235 | doi = 10.1016/S0014-5793(00)01320-X | bibcode = 2000FEBSL.470..207W }}</ref>
* [[Nucleolar protein, member A1|GAR1]],<ref name=pmid11509230/>
* [[Survival of motor neuron protein-interacting protein 1|GEMIN2]],<ref name=pmid10942426/><ref name=pmid9323129>{{cite journal | vauthors = Liu Q, Fischer U, Wang F, Dreyfuss G | title = The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins | journal = Cell | volume = 90 | issue = 6 | pages = 1013–21 | date = September 1997 | pmid = 9323129 | doi = 10.1016/S0092-8674(00)80367-0 | doi-access = free }}</ref>
* [[GEMIN4]],<ref name=pmid10942426>{{cite journal | vauthors = Meister G, Bühler D, Laggerbauer B, Zobawa M, Lottspeich F, Fischer U | title = Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins | journal = Human Molecular Genetics | volume = 9 | issue = 13 | pages = 1977–86 | date = August 2000 | pmid = 10942426 | doi = 10.1093/hmg/9.13.1977 | doi-access = free }}</ref>
* [[GEMIN5]],<ref name=pmid11714716>{{cite journal | vauthors = Gubitz AK, Mourelatos Z, Abel L, Rappsilber J, Mann M, Dreyfuss G | title = Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins | journal = The Journal of Biological Chemistry | volume = 277 | issue = 7 | pages = 5631–6 | date = February 2002 | pmid = 11714716 | doi = 10.1074/jbc.M109448200 | doi-access = free }}</ref>
* [[Gem-associated protein 7|GEMIN7]],<ref name=pmid12065586>{{cite journal | vauthors = Baccon J, Pellizzoni L, Rappsilber J, Mann M, Dreyfuss G | title = Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex | journal = The Journal of Biological Chemistry | volume = 277 | issue = 35 | pages = 31957–62 | date = August 2002 | pmid = 12065586 | doi = 10.1074/jbc.M203478200 | doi-access = free }}</ref><ref name=pmid11748230>{{cite journal | vauthors = Pellizzoni L, Baccon J, Rappsilber J, Mann M, Dreyfuss G | title = Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component | journal = The Journal of Biological Chemistry | volume = 277 | issue = 9 | pages = 7540–5 | date = March 2002 | pmid = 11748230 | doi = 10.1074/jbc.M110141200 | doi-access = free }}</ref>
* [[HNRNPR]],<ref name=pmid11574476>{{cite journal | vauthors = Mourelatos Z, Abel L, Yong J, Kataoka N, Dreyfuss G | title = SMN interacts with a novel family of hnRNP and spliceosomal proteins | journal = The EMBO Journal | volume = 20 | issue = 19 | pages = 5443–52 | date = October 2001 | pmid = 11574476 | pmc = 125643 | doi = 10.1093/emboj/20.19.5443 }}</ref><ref name=pmid11773003>{{cite journal | vauthors = Rossoll W, Kröning AK, Ohndorf UM, Steegborn C, Jablonka S, Sendtner M | title = Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons? | journal = Human Molecular Genetics | volume = 11 | issue = 1 | pages = 93–105 | date = January 2002 | pmid = 11773003 | doi = 10.1093/hmg/11.1.93 | doi-access = free }}</ref>
* [[KPNB1]],<ref name=pmid12095920>{{cite journal | vauthors = Narayanan U, Ospina JK, Frey MR, Hebert MD, Matera AG | title = SMN, the spinal muscular atrophy protein, forms a pre-import snRNP complex with snurportin1 and importin beta | journal = Human Molecular Genetics | volume = 11 | issue = 15 | pages = 1785–95 | date = July 2002 | pmid = 12095920 | pmc = 1630493 | doi = 10.1093/hmg/11.15.1785 }}</ref>
* [[P53]],<ref name=pmid11704667>{{cite journal | vauthors = Young PJ, Day PM, Zhou J, Androphy EJ, Morris GE, Lorson CL | title = A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy | journal = The Journal of Biological Chemistry | volume = 277 | issue = 4 | pages = 2852–9 | date = January 2002 | pmid = 11704667 | doi = 10.1074/jbc.M108769200 | doi-access = free }}</ref>
* [[Small nuclear ribonucleoprotein D1|SNRPD1]],<ref name=pmid10942426/><ref name=pmid9323129/><ref name=pmid10851237>{{cite journal | vauthors = Friesen WJ, Dreyfuss G | title = Specific sequences of the Sm and Sm-like (Lsm) proteins mediate their interaction with the spinal muscular atrophy disease gene product (SMN) | journal = The Journal of Biological Chemistry | volume = 275 | issue = 34 | pages = 26370–5 | date = August 2000 | pmid = 10851237 | doi = 10.1074/jbc.M003299200 | doi-access = free }}</ref> and
* [[Small nuclear ribonucleoprotein D2|SNRPD2]].<ref name=pmid10942426/>
{{Div col end}}


==Evolutionary conservation==
==Evolutionary conservation==
SMN is evolutionary conserved including the [[Fungi]] kingdom, though only fungal organisms with a great number of [[intron]]s has the smn gene (or the [[spf30]] paralogue). Surprisingly, these are filamentous fungus which have [[mycelia]], so suggesting [[Analogy (biology)|analogy]] to the neuronal axons.<ref>{{cite journal | vauthors = Mier P, Pérez-Pulido AJ | title = Fungal Smn and Spf30 homologues are mainly present in filamentous fungi and genomes with many introns: implications for spinal muscular atrophy | journal = Gene | volume = 491 | issue = 2 | pages = 135–41 | date = January 2012 | pmid = 22020225 | doi = 10.1016/j.gene.2011.10.006 }}</ref>
SMN is evolutionarily conserved including the [[Fungi]] kingdom, though only fungal organisms with a great number of [[intron]]s have the ''Smn'' gene (or the [[splicing factor]] [[spf30]] [[paralogue]]). Surprisingly, these are filamentous fungus which have [[mycelia]], so suggesting [[Analogy (biology)|analogy]] to the neuronal axons.<ref>{{cite journal | vauthors = Mier P, Pérez-Pulido AJ | title = Fungal Smn and Spf30 homologues are mainly present in filamentous fungi and genomes with many introns: implications for spinal muscular atrophy | journal = Gene | volume = 491 | issue = 2 | pages = 135–41 | date = January 2012 | pmid = 22020225 | doi = 10.1016/j.gene.2011.10.006 | url = https://zenodo.org/record/897704 }}</ref>


== See also ==
== See also ==
Line 40: Line 67:


[[Category:Motor system]]
[[Category:Motor system]]
[[Category:Spinal muscular atrophy]]


{{Biochem-stub}}

Latest revision as of 23:04, 9 June 2024

Survival motor neuron protein
Tudor domain from human SMN. PDB 1g5v[1]
Identifiers
SymbolSMN
PfamPF06003
Pfam clanCL0049
InterProIPR010304
SCOP21mhn / SCOPe / SUPFAM
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary

Survival of motor neuron or survival motor neuron (SMN) is a protein that in humans is encoded by the SMN1 and SMN2 genes.

SMN is found in the cytoplasm of all animal cells and also in the nuclear gems. It functions in transcriptional regulation, telomerase regeneration and cellular trafficking.[2] SMN deficiency, primarily due to mutations in SMN1, results in widespread splicing defects, especially in spinal motor neurons, and is one cause of spinal muscular atrophy. Research also showed a possible role of SMN in neuronal migration and/or differentiation.[3]

Function

[edit]

The SMN protein contains GEMIN2-binding, Tudor and YG-Box domains.[4] It localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as GEMIN2 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein.[5]

SMN complex

[edit]

SMN complex refers to the entire multi-protein complex involved in the assembly of snRNPs, the essential components of spliceosomal machinery.[6] The complex, apart from the "proper" survival of motor neuron protein, includes at least six other proteins (gem-associated protein 2, 3, 4, 5, 6 and 7.[6]

Interactions

[edit]

SMN has been shown to interact with:

Evolutionary conservation

[edit]

SMN is evolutionarily conserved including the Fungi kingdom, though only fungal organisms with a great number of introns have the Smn gene (or the splicing factor spf30 paralogue). Surprisingly, these are filamentous fungus which have mycelia, so suggesting analogy to the neuronal axons.[25]

See also

[edit]

References

[edit]
  1. ^ Selenko P, Sprangers R, Stier G, Bühler D, Fischer U, Sattler M (January 2001). "SMN tudor domain structure and its interaction with the Sm proteins". Nature Structural Biology. 8 (1): 27–31. doi:10.1038/83014. PMID 11135666.
  2. ^ Singh NN, Shishimorova M, Cao LC, Gangwani L, Singh RN (2009). "A short antisense oligonucleotide masking a unique intronic motif prevents skipping of a critical exon in spinal muscular atrophy". RNA Biology. 6 (3): 341–50. doi:10.4161/rna.6.3.8723. PMC 2734876. PMID 19430205.
  3. ^ Giavazzi A, Setola V, Simonati A, Battaglia G (March 2006). "Neuronal-specific roles of the survival motor neuron protein: evidence from survival motor neuron expression patterns in the developing human central nervous system". Journal of Neuropathology and Experimental Neurology. 65 (3): 267–77. doi:10.1097/01.jnen.0000205144.54457.a3. PMID 16651888.
  4. ^ Martin R, Gupta K, Ninan NS, Perry K, Van Duyne GD (November 2012). "The survival motor neuron protein forms soluble glycine zipper oligomers". Structure. 20 (11): 1929–39. doi:10.1016/j.str.2012.08.024. PMC 3519385. PMID 23022347.
  5. ^ "Entrez Gene: SMN1 survival of motor neuron 1, telomeric".
  6. ^ a b Gubitz AK, Feng W, Dreyfuss G (May 2004). "The SMN complex". Experimental Cell Research. 296 (1): 51–6. doi:10.1016/j.yexcr.2004.03.022. PMID 15120993.
  7. ^ Iwahashi H, Eguchi Y, Yasuhara N, Hanafusa T, Matsuzawa Y, Tsujimoto Y (November 1997). "Synergistic anti-apoptotic activity between Bcl-2 and SMN implicated in spinal muscular atrophy". Nature. 390 (6658): 413–7. Bibcode:1997Natur.390..413I. doi:10.1038/37144. PMID 9389483.
  8. ^ Hebert MD, Shpargel KB, Ospina JK, Tucker KE, Matera AG (September 2002). "Coilin methylation regulates nuclear body formation". Developmental Cell. 3 (3): 329–37. doi:10.1016/S1534-5807(02)00222-8. PMID 12361597.
  9. ^ Hebert MD, Szymczyk PW, Shpargel KB, Matera AG (October 2001). "Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein". Genes & Development. 15 (20): 2720–9. doi:10.1101/gad.908401. PMC 312817. PMID 11641277.
  10. ^ a b c d e Meister G, Bühler D, Laggerbauer B, Zobawa M, Lottspeich F, Fischer U (August 2000). "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins". Human Molecular Genetics. 9 (13): 1977–86. doi:10.1093/hmg/9.13.1977. PMID 10942426.
  11. ^ Mourelatos Z, Dostie J, Paushkin S, Sharma A, Charroux B, Abel L, Rappsilber J, Mann M, Dreyfuss G (March 2002). "miRNPs: a novel class of ribonucleoproteins containing numerous microRNAs". Genes & Development. 16 (6): 720–8. doi:10.1101/gad.974702. PMC 155365. PMID 11914277.
  12. ^ Charroux B, Pellizzoni L, Perkinson RA, Shevchenko A, Mann M, Dreyfuss G (December 1999). "Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems". The Journal of Cell Biology. 147 (6): 1181–94. doi:10.1083/jcb.147.6.1181. PMC 2168095. PMID 10601333.
  13. ^ Pellizzoni L, Charroux B, Rappsilber J, Mann M, Dreyfuss G (January 2001). "A functional interaction between the survival motor neuron complex and RNA polymerase II". The Journal of Cell Biology. 152 (1): 75–85. doi:10.1083/jcb.152.1.75. PMC 2193649. PMID 11149922.
  14. ^ a b Pellizzoni L, Baccon J, Charroux B, Dreyfuss G (July 2001). "The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1". Current Biology. 11 (14): 1079–88. Bibcode:2001CBio...11.1079P. doi:10.1016/S0960-9822(01)00316-5. PMID 11509230.
  15. ^ Williams BY, Hamilton SL, Sarkar HK (March 2000). "The survival motor neuron protein interacts with the transactivator FUSE binding protein from human fetal brain". FEBS Letters. 470 (2): 207–10. Bibcode:2000FEBSL.470..207W. doi:10.1016/S0014-5793(00)01320-X. PMID 10734235.
  16. ^ a b Liu Q, Fischer U, Wang F, Dreyfuss G (September 1997). "The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins". Cell. 90 (6): 1013–21. doi:10.1016/S0092-8674(00)80367-0. PMID 9323129.
  17. ^ Gubitz AK, Mourelatos Z, Abel L, Rappsilber J, Mann M, Dreyfuss G (February 2002). "Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins". The Journal of Biological Chemistry. 277 (7): 5631–6. doi:10.1074/jbc.M109448200. PMID 11714716.
  18. ^ Baccon J, Pellizzoni L, Rappsilber J, Mann M, Dreyfuss G (August 2002). "Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex". The Journal of Biological Chemistry. 277 (35): 31957–62. doi:10.1074/jbc.M203478200. PMID 12065586.
  19. ^ Pellizzoni L, Baccon J, Rappsilber J, Mann M, Dreyfuss G (March 2002). "Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component". The Journal of Biological Chemistry. 277 (9): 7540–5. doi:10.1074/jbc.M110141200. PMID 11748230.
  20. ^ Mourelatos Z, Abel L, Yong J, Kataoka N, Dreyfuss G (October 2001). "SMN interacts with a novel family of hnRNP and spliceosomal proteins". The EMBO Journal. 20 (19): 5443–52. doi:10.1093/emboj/20.19.5443. PMC 125643. PMID 11574476.
  21. ^ Rossoll W, Kröning AK, Ohndorf UM, Steegborn C, Jablonka S, Sendtner M (January 2002). "Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons?". Human Molecular Genetics. 11 (1): 93–105. doi:10.1093/hmg/11.1.93. PMID 11773003.
  22. ^ Narayanan U, Ospina JK, Frey MR, Hebert MD, Matera AG (July 2002). "SMN, the spinal muscular atrophy protein, forms a pre-import snRNP complex with snurportin1 and importin beta". Human Molecular Genetics. 11 (15): 1785–95. doi:10.1093/hmg/11.15.1785. PMC 1630493. PMID 12095920.
  23. ^ Young PJ, Day PM, Zhou J, Androphy EJ, Morris GE, Lorson CL (January 2002). "A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy". The Journal of Biological Chemistry. 277 (4): 2852–9. doi:10.1074/jbc.M108769200. PMID 11704667.
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