The DOI matches its Crossref record; 41 of the 41 references it cites were found in a registry or an index.
The Spinal Muscular Atrophy Disease Gene Product, SMN, and Its Associated Protein SIP1 Are in a Complex with Spliceosomal snRNP Proteins
Open accessAbstract
Spinal muscular atrophy (SMA), one of the most common fatal autosomal recessive diseases, is characterized by degeneration of motor neurons and muscular atrophy. The SMA disease gene, termed Survival of Motor Neurons (SMN), is deleted or mutated in over 98% of SMA patients. The function of the SMN protein is unknown. We found that SMN is tightly associated with a novel protein, SIP1, and together they form a specific complex with several spliceosomal snRNP proteins. SMN interacts directly with several of the snRNP Sm core proteins, including B, D1-3, and E. Interestingly, SIP1 has significant sequence similarity with Brr1, a yeast protein critical for snRNP biogenesis. These findings suggest a role for SMN and SIP1 in spliceosomal snRNP biogenesis and function and provide a likely molecular mechanism for the cause of SMA.
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Identification and characterization of a spinal muscular atrophy-determining gene
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hnRNP PROTEINS AND THE BIOGENESIS OF mRNA
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Self-citationCorrelation between severity and SMN protein level in spinal muscular atrophy
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Self-citationA novel nuclear structure containing the survival of motor neurons protein.
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The SMN–SIP1 Complex Has an Essential Role in Spliceosomal snRNP Biogenesis
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Cited by
Induced pluripotent stem cells from a spinal muscular atrophy patient
Ebert, A. D., Yu, J., Rose, F. F., Mattis, V. B., Lorson, C. L., Thomson, J. A., & Svendsen, C. N. (2009) · Nature · doi:10.1038/nature07677 · Cited by 1,441
Nucleocytoplasmic transport: the soluble phase
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miRNPs: a novel class of ribonucleoproteins containing numerous microRNAs
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Self-citationA day in the life of the spliceosome
Matera, A. G., & Wang, Z. (2014) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3742 · Cited by 1,074
Ishov, A. M., Sotnikov, A. G., Negorev, D., Vladimirova, O. V., Neff, N., Kamitani, T., Yeh, E. T. H., Strauss, J. F., & Maul, G. G. (1999) · The Journal of Cell Biology · doi:10.1083/jcb.147.2.221 · Cited by 832
Metadata
- Authors
- Liu, Q., Fischer, U., Wang, F., & Dreyfuss, G.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 1997-09
- Volume
- 90
- Issue
- 6
- Pages
- 1013-1021
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2096650232
- Cited by
- 657
- References
- 41
- FWCI
- 30.59
- Type
- journal article
- Language
- en
- Open access
- hybrid
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