Survival of motor neuron-related-splicing factor 30 is a protein that in humans is encoded by the SMNDC1 gene. GRCh38: Ensembl ... "Characterization of a gene encoding survival motor neuron (SMN)-related protein, a constituent of the spliceosome complex". Hum ... "Entrez Gene: SMNDC1 survival motor neuron domain containing 1". Rappsilber J, Ajuh P, Lamond AI, Mann M (2001). "SPF30 is an ... 2007). "Large-scale mapping of human protein-protein interactions by mass spectrometry". Mol. Syst. Biol. 3 (1): 89. doi: ...

Hua Y, Zhou J (2004). "Survival motor neuron protein facilitates assembly of stress granules". FEBS Lett. 572 (1-3): 69-74. doi ... The protein encoded by this gene is a member of a family of RNA-binding proteins, has three RNA recognition motifs (RRMs), and ... 2000). "Expression of the RNA-binding protein TIAR is increased in neurons after ischemic cerebral injury". J. Neurosci. Res. ... 2002). "Cell proteins TIA-1 and TIAR interact with the 3' stem-loop of the West Nile virus complementary minus-strand RNA and ...

... (GEMIN2), also called survival of motor neuron protein-interacting protein 1 (SIP1), is a protein that ... "Entrez Gene: SIP1 survival of motor neuron protein interacting protein 1". Mourelatos Z, Dostie J, Paushkin S, Sharma A, ... May 2003). "Protein phosphatase 4 interacts with the Survival of Motor Neurons complex and enhances the temporal localisation ... "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of ...

"The oral splicing modifier RG7800 increases full length survival of motor neuron 2 mRNA and survival of motor neuron protein: ... the SMN1 gene codes the survival of motor neuron protein (SMN) which, as its name says, plays a crucial role in survival of ... a protein necessary for survival of motor neurons. Loss of these neurons in the spinal cord prevents signalling between the ... Neuroprotective drugs aim at enabling the survival of motor neurons even with low levels of SMN protein. Olesoxime was a ...

"Protein phosphatase 4 interacts with the Survival of Motor Neurons complex and enhances the temporal localisation of snRNPs". J ... "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of ... "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of ... "Protein phosphatase 4 interacts with the Survival of Motor Neurons complex and enhances the temporal localisation of snRNPs". J ...

2001). "A functional interaction between the survival motor neuron complex and RNA polymerase II". J. Cell Biol. 152 (1): 75-85 ... 2000). "A mammalian germ cell-specific RNA-binding protein interacts with ubiquitously expressed proteins involved in splice ... RNA-binding motif protein, Y chromosome, family 1 member A1/C is a protein that in humans is encoded by the RBMY1A1 gene. This ... Multiple copies of this gene are found in the AZFb azoospermia factor region of chromosome Y and the encoded protein is thought ...

A 2004 study using high-throughput screening found indoprofen to increase production of the survival of motor neuron protein, ... November 2004). "Indoprofen upregulates the survival motor neuron protein through a cyclooxygenase-independent mechanism". ... 1-Oxo-2-substituted isoindoline derivatives". Arzneimittel-Forschung. 23 (8): 1090-100. doi:10.1002/chin.197344288. PMID ... Reduction of the nitro group in arylpropionic acid (1) gives the corresponding aniline (2). Reaction of the intermediate with ...

"The Ewing's sarcoma protein interacts with the Tudor domain of the survival motor neuron protein". Molecular Brain Research. ... and FUS proteins are members of the FET protein family of RNA binding proteins. They are partners in various fusion proteins ... The fusion proteins found in the neoplastic cells of EMC consist of NR4A3 in >90% of cases partnered with EWRS1 in >75% of ... EMC patients have a long-term clinical course with a survival rate of 5 years in 90% of patients, 10 years at 70% and 15 years ...

... protein shortage. This is due to the deletion or mutation of Survival of Motor Neuron 1 (SMN1). Due to the faulty SMN1 gene, ... SMN2 cannot produce sufficient full-length protein for the motor neurons to signal muscles due to sequence differences and the ... SMA patients do not have enough SMN protein. SMA patients must depend on Survivor of Motor Neurons 2 (SMN2), a gene that all ... is a hereditary degenerative disease of the central nervous system resulting from a spinal motor neuron (SMN) ...

"The survival motor neuron protein interacts with the transactivator FUSE binding protein from human fetal brain". FEBS Lett. ... "The survival motor neuron protein interacts with the transactivator FUSE binding protein from human fetal brain". FEBS Lett. ... This protein has been shown to function as an ATP-dependent DNA helicase. Far upstream element-binding protein 1 has been shown ... 2005). "A human protein-protein interaction network: a resource for annotating the proteome". Cell. 122 (6): 957-68. doi: ...

"The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1". Curr. Biol. 11 (14): 1079 ... "The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1". Curr. Biol. 11 (14): 1079 ... The H/ACA snoRNPs also include the DKC1, NOLA2 and NOLA3 proteins. These four H/ACA snoRNP proteins localize to the dense ... These four H/ACA snoRNP proteins are also components of the telomerase complex. The encoded protein of this gene contains two ...

Liu Q, Dreyfuss G (July 1996). "A novel nuclear structure containing the survival of motor neurons protein". The EMBO Journal. ... "The survival motor neuron protein in spinal muscular atrophy". Human Molecular Genetics. 6 (8): 1205-14. doi:10.1093/hmg/6.8. ... "Heterogeneity of subcellular localization and electrophoretic mobility of survival motor neuron (SMN) protein in mammalian ... Survival of motor neuron 1 (SMN1), also known as component of gems 1 or GEMIN1, is a gene that encodes the SMN protein in ...

This gene encodes a DEAD box protein, which has an ATPase activity and is a component of the survival of motor neuron (SMN) ... May 2003). "Protein phosphatase 4 interacts with the Survival of Motor Neurons complex and enhances the temporal localisation ... May 2003). "Protein phosphatase 4 interacts with the Survival of Motor Neurons complex and enhances the temporal localisation ... "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of ...

The 3′ stem structure is necessary for recognition by the survival of motor neuron (SMN) protein. This complex assembles the ... Spinal muscular atrophy - Mutations in the survival motor neuron-1 (SMN1) gene result in the degeneration of spinal motor ... repeatable method of RNA processing is necessary to ensure cell survival. The spliceosome is a large, protein-RNA complex that ... including Sm proteins, a family of nuclear proteins). The most common human snRNA components of these complexes are known, ...

"Interaction between the small-nuclear-RNA cap hypermethylase and the spinal muscular atrophy protein, survival of motor neuron ... Zhu Y, Qi C, Cao WQ, Yeldandi AV, Rao MS, Reddy JK (Aug 2001). "Cloning and characterization of PIMT, a protein with a ... Enünlü I, Pápai G, Cserpán I, Udvardy A, Jeang KT, Boros I (Sep 2003). "Different isoforms of PRIP-interacting protein with ... TGS1 has been shown to interact with: CREB-binding protein, and EED, EP300, MED1, and NCOA6. GRCh38: Ensembl release 89: ...

As a treatment for SMA, branaplam increases the amount of functional survival of motor neuron protein produced by the SMN2 gene ... In October 2020, Novartis announced that branaplam lowers the level of huntingtin protein, which is one of the major ... It was studied since 2014 in a clinical trial in children with SMA type 1 until the programme was discontinued in 2021. ... "An Open Label Study of LMI070 in Type 1 Spinal Muscular Atrophy (SMA)". ClinicalTrials.gov. Retrieved 2017-03-10. "Novartis ...

... or survival motor neuron (SMN) is a protein that in humans is encoded by the SMN1 and SMN2 genes. SMN ... "Neuronal-specific roles of the survival motor neuron protein: evidence from survival motor neuron expression patterns in the ... survival of motor neuron protein, includes at least six other proteins (gem-associated protein 2, 3, 4, 5, 6 and 7. SMN has ... "The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1". Current Biology. 11 (14 ...

Liu Q, Dreyfuss G (1996). "A novel nuclear structure containing the survival of motor neurons protein". EMBO J. 15 (14): 3555- ... "Major cell surface-located protein substrates of an ecto-protein kinase are homologs of known nuclear proteins". Biochemistry. ... The hnRNP proteins have distinct nucleic acid binding properties. The protein encoded by this gene contains a RNA binding ... The hnRNPs are RNA binding proteins that form complexes with heterogeneous nuclear RNA (hnRNA). These proteins are associated ...

... a novel component of the survival of motor neuron complex". The Journal of Biological Chemistry. 277 (35): 31957-62. doi: ... Syntaxin-binding protein 3 is a protein that in humans is encoded by the STXBP3 gene. Syntaxin binding protein 3 has been shown ... "Entrez Gene: STXBP3 syntaxin binding protein 3". Schraw TD, Lemons PP, Dean WL, Whiteheart SW (August 2003). "A role for Sec1/ ... Hodgkinson CP, Mander A, Sale GJ (June 2005). "Identification of 80K-H as a protein involved in GLUT4 vesicle trafficking". The ...

As SMN protein generally promotes the survival of motor neurons, mutations in SMN1 results in slow degeneration motor neurons ... Neurodengenerative diseases of motor neurons can cause degeneration of motor neurons involved in voluntary muscle control such ... is a motor neuron disease that involves neurogeneration. All skeletal muscles in the body are controlled by motor neurons that ... See the Motor Neuron Fact Sheet for details regarding other motor neuron diseases. Neurodegenerative diseases of the central ...

In primary cortical and motor neurons, progranulin regulates neuronal outgrowth and survival. In primary motor neurons, ... The structure of the granulin protein is similar to the structure of proteins from protein families that consist of hormones, ... Granulin is a protein that in humans is encoded by the GRN gene. Each granulin protein is cleaved from the precursor ... November 2010). "Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin". Neuron. ...

Pellizzoni L, Baccon J, Rappsilber J, Mann M, Dreyfuss G (March 2002). "Purification of native survival of motor neurons ... Fury MG, Zhang W, Christodoulopoulos I, Zieve GW (November 1997). "Multiple protein: protein interactions between the snRNP ... Fury MG, Zhang W, Christodoulopoulos I, Zieve GW (November 1997). "Multiple protein: protein interactions between the snRNP ... and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins". Cell. 90 (6): 1013-21. doi:10.1016/S0092- ...

CNTF: Ciliary neurotrophic factor is another protein that acts as a survival factor for motor neurons. CNTF acts via a receptor ... Motor neurons innervating the same twitch muscle fibers are thought to maintain synchronous activity which allows both neurons ... The survival of neurons is regulated by survival factors, called trophic factors. The neurotrophic hypothesis was formulated by ... Synaptic communication between neurons leads to the establishment of functional neural circuits that mediate sensory and motor ...

Specifically, the survival motor neuron (SMN) protein is a Tudor domain protein that mediates the assembly of snRNPs (small ... The structurally characterized Tudor domain in human SMN (survival of motor neuron) is a strongly bent anti-parallel β-sheet ... These proteins contain RNA-binding motifs to target RNAs, or bind to dimethylated arginines of proteins bound to RNAs. These ... The proteins TP53BP1 (Tumor suppressor p53-binding protein 1) and its fission yeast homolog Crb2 and JMJD2A (Jumonji domain ...

"The Gemin6-Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein-like structure". Structure. 13 (6): ... Pellizzoni L, Baccon J, Rappsilber J, Mann M, Dreyfuss G (2002). "Purification of native survival of motor neurons complexes ... "snRNP Sm proteins share two evolutionarily conserved sequence motifs which are involved in Sm protein-protein interactions". ... Fury MG, Zhang W, Christodoulopoulos I, Zieve GW (1997). "Multiple protein: protein interactions between the snRNP common core ...

... has also been found to significantly increase in vitro the survival of motor neuron (SMN) protein levels in cells ... Plasma protein binding is about 90%. The substance is metabolised mainly through oxidation by the liver enzyme CYP3A4 and to a ... October 2009). "LBH589 induces up to 10-fold SMN protein levels by several independent mechanisms and is effective even in ... 3 (1): 33-38. Simons J (27 April 2013). "Scientists on brink of HIV cure". The Telegraph. Archived from the original on 27 ...

2000). "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset ... "Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of ... 2002). "Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel component". J. Biol ... 2002). "Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex". J. Biol. ...

The opposite effect is seen with interactions between the survival of motor neurons protein and the snRNP proteins SmD1, SmD3 ... C-terminal protein methylation regulates the assembly of protein phosphatase. Methylation of the protein phosphatase 2A ... The arginine methylation of proteins can either inhibit or promote protein-protein interactions depending on the type of ... GTP-binding proteins, nuclear lamins and certain protein kinases. Many of these proteins participate in cell signaling, and ...

November 2010). "WRAP53 is essential for Cajal body formation and for targeting the survival of motor neuron complex to Cajal ... WRAP53β facilitates protein-protein and protein-RNA interactions and directs factors to nuclear organelles Cajal bodies, to ... May 2012). "Reorganization of Cajal bodies and nucleolar targeting of coilin in motor neurons of type I spinal muscular atrophy ... The WRAP53 gene also encodes a protein termed WRAP53β (alias WRAP53 or WDR79 or TCAB1), which belongs to the WD40 protein ...

"The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1". Current Biology. 11 (14 ... "The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1". Current Biology. 11 (14 ... Liu Q, Dreyfuss G (Jul 1996). "A novel nuclear structure containing the survival of motor neurons protein". The EMBO Journal. ... The 15.5-kDa protein has sequence similarity to other RNA-binding proteins such as ribosomal proteins S12, L7a, and L30 and the ...