SMN2 and DHX9

Number of citations: 78 (PubMedID 11149922)
Data Source: HPRD (in vitro, in vivo)

		SMN2	DHX9
Name		survival of motor neuron 2, centromeric	DExH-box helicase 9
Structure
GO Annotations	Cellular Component	Nucleus Nucleoplasm Cytoplasm Cytosol Cajal Body Nuclear Body Z Disc Axon SMN Complex SMN-Sm Protein Complex Cytoplasmic Ribonucleoprotein Granule Cell Projection Neuron Projection Perikaryon Gemini Of Cajal Bodies	Nucleus Nucleoplasm Perichromatin Fibrils Nucleolus Cytoplasm Centrosome Cytosol Cytoskeleton Actin Cytoskeleton Membrane RISC Complex Nuclear Body Protein-containing Complex Cytoplasmic Ribonucleoprotein Granule RISC-loading Complex CRD-mediated MRNA Stability Complex Nuclear Stress Granule Ribonucleoprotein Complex
	Molecular Function	RNA Binding Protein Binding Identical Protein Binding	Nucleotide Binding RNA Polymerase II Cis-regulatory Region Sequence-specific DNA Binding RNA Polymerase II Complex Binding Regulatory Region RNA Binding Nucleic Acid Binding DNA Binding DNA Helicase Activity DNA Replication Origin Binding Double-stranded DNA Binding Single-stranded DNA Binding Transcription Coregulator Activity Transcription Coactivator Activity RNA Binding RNA Helicase Activity Double-stranded RNA Binding Single-stranded RNA Binding MRNA Binding Helicase Activity Protein Binding ATP Binding Hydrolase Activity ATP Hydrolysis Activity Ribonucleoside Triphosphate Phosphatase Activity Chromatin DNA Binding 3'-5' DNA/RNA Helicase Activity 3'-5' RNA Helicase Activity SiRNA Binding RNA Stem-loop Binding Ribosome Binding 3'-5' DNA Helicase Activity Triplex DNA Binding Metal Ion Binding Nucleoside Triphosphate Diphosphatase Activity RNA Polymerase II-specific DNA-binding Transcription Factor Binding Importin-alpha Family Protein Binding RNA Polymerase Binding Catalytic Activity, Acting On A Nucleic Acid RISC Complex Binding Single-stranded 3'-5' DNA Helicase Activity Sequence-specific MRNA Binding Promoter-specific Chromatin Binding
	Biological Process	Spliceosomal Complex Assembly RNA Splicing, Via Transesterification Reactions Spliceosomal SnRNP Assembly DNA-templated Transcription Termination MRNA Processing Nervous System Development RNA Splicing	Alternative MRNA Splicing, Via Spliceosome Osteoblast Differentiation Immune System Process DNA Replication Chromatin Organization DNA-templated Transcription Termination Regulation Of Transcription By RNA Polymerase II MRNA Processing Regulation Of Translation Inflammatory Response RNA Splicing Regulatory NcRNA-mediated Gene Silencing Positive Regulation Of Interferon-alpha Production Positive Regulation Of Interferon-beta Production Positive Regulation Of Interleukin-18 Production Positive Regulation Of Interleukin-6 Production Positive Regulation Of Tumor Necrosis Factor Production MiRNA-mediated Post-transcriptional Gene Silencing DNA-templated Viral Transcription Innate Immune Response Positive Regulation Of Innate Immune Response Positive Regulation Of DNA Repair Positive Regulation Of DNA Replication Positive Regulation Of Transcription By RNA Polymerase II Positive Regulation Of RNA Export From Nucleus Positive Regulation Of Fibroblast Proliferation Rhythmic Process Positive Regulation Of Viral Transcription Regulation Of MRNA Processing Regulation Of Defense Response To Virus By Host Positive Regulation Of Inflammatory Response MRNA Transport Positive Regulation Of NF-kappaB Transcription Factor Activity Positive Regulation Of Response To Cytokine Stimulus Protein-containing Complex Assembly Pyroptotic Inflammatory Response RISC Complex Assembly CRD-mediated MRNA Stabilization Cellular Response To Exogenous DsRNA Negative Regulation Of Nuclear-transcribed MRNA Catabolic Process, Deadenylation-dependent Decay Protein Localization To Cytoplasmic Stress Granule Regulation Of Cytoplasmic Translation Positive Regulation Of Cytoplasmic Translation
Pathways		snRNP Assembly SARS-CoV-2 modulates host translation machinery	RIP-mediated NFkB activation via ZBP1 DEx/H-box helicases activate type I IFN and inflammatory cytokines production mRNA Splicing - Major Pathway PKR-mediated signaling
Drugs		Nusinersen	None
Diseases		Spinal muscular atrophy (SMA), including: SMA type I (SMA1) / Werdning-Hoffman disease; SMA type II (SMA2); SMA type III (SMA3) / Kugeleberg-Welander disease; SMA type IV (SMA4); X-linked SMA 2 (SMAX2); X-linked distal SMA 3 (DSMAX); Distal SMA autosomal recessive type 4 (DSMA4); Congenital distal spinal muscular atrophy (SMAL); SMA proximal adult autosomal dominant (SMAPAD)	None
GWAS		No GWAS studies recorded	No GWAS studies recorded
Novel Interactors		None recorded	2 novel interactors: JAK1 TYK2
Interacting Genes		37 interacting genes: BLOC1S6 BYSL CHTOP DDX20 DHX9 EHHADH FAM9B FBL FNDC11 GAR1 GEMIN2 HNRNPUL1 IQUB KRTAP19-6 KRTAP19-7 KRTAP21-2 KRTAP6-1 KRTAP6-2 MAGED1 MBD2 POLR1C POLR2A PPIG SMN1 SNRPB SNRPB2 SNRPD1 SNRPD2 SNRPD3 SNRPE SNRPF SNRPG SNU13 SPANXN2 TIAL1 USP9X VPS28	44 interacting genes: ACTA1 AKAP8L APBB1 BRCA1 DDX20 DUX4 GEMIN2 GEMIN4 GTF2B HDLBP HNRNPC HNRNPK IL7R ILF3 JUN KHDRBS1 LINC01016 LINC01554 MBD2 NXF1 OGA OGT PCNA POLR2A PRMT1 PRPF40A PRPF8 PTEN RBM4 RELA RNF10 RNF168 SMN1 SMN2 SNRPB SNRPB2 SRPK1 SSRP1 STAU1 TBP TOP2A UBE2I USP7 YWHAQ
Entrez ID		6607	1660
HPRD ID		09036	04386
Ensembl ID		ENSG00000205571	ENSG00000135829
Uniprot IDs		B4DP61 E7EQZ4 Q16637	B3KU66 Q08211
PDB IDs		1G5V 1MHN 2LEH 4A4E 4A4G 4GLI 4QQ6 5XJL 5XJQ 5XJR 5XJS 5XJT 5XJU 7W2P 7W30	3LLM 3VYX 3VYY 8SZP
GO Terms Enriched among Interactors		SMN-Sm Protein Complex Spliceosomal SnRNP Assembly 7-methylguanosine Cap Hypermethylation Spliceosomal Complex Assembly U1 SnRNP U4 SnRNP Methylosome Protein-RNA Complex Assembly U2-type Prespliceosome Assembly RNA Splicing, Via Transesterification Reactions RNA Splicing U2 SnRNP U2-type Precatalytic Spliceosome U2-type Spliceosomal Complex U2-type Catalytic Step 2 Spliceosome Small Nuclear Ribonucleoprotein Complex U5 SnRNP U4/U6 X U5 Tri-snRNP Complex RNA Processing U12-type Spliceosomal Complex MRNA Processing PICln-Sm Protein Complex MRNA Splicing, Via Spliceosome Ribonucleoprotein Complex MRNA Metabolic Process U7 SnRNP Precatalytic Spliceosome Spliceosomal Complex RNA Methylation RNA Metabolic Process Catalytic Step 2 Spliceosome RNA Binding RNA Modification Macromolecule Methylation Nucleic Acid Metabolic Process Nucleobase-containing Compound Metabolic Process Methylation Nucleoplasm Telomerase Holoenzyme Complex Protein-containing Complex Assembly Spliceosomal Tri-snRNP Complex Cellular Component Assembly Protein-containing Complex Organization Cytosol SMN Complex Gemini Of Cajal Bodies DNA-templated Transcription Termination Telomerase RNA Binding Intermediate Filament Histone Pre-mRNA DCP Binding	RNA Splicing Nucleoplasm MRNA Metabolic Process MRNA Processing Nucleic Acid Metabolic Process Nucleus RNA Binding SMN-Sm Protein Complex Nucleobase-containing Compound Metabolic Process Spliceosomal SnRNP Assembly Macromolecule Metabolic Process RNA Processing SMN Complex Gemini Of Cajal Bodies RNA Metabolic Process RNA Splicing, Via Transesterification Reactions Regulation Of RNA Metabolic Process Regulation Of Nucleobase-containing Compound Metabolic Process Chromatin Organization Protein-RNA Complex Assembly Spliceosomal Complex Assembly Regulation Of Gene Expression Positive Regulation Of Macromolecule Biosynthetic Process Regulation Of Macromolecule Biosynthetic Process Regulation Of DNA-templated Transcription Nuclear Body Regulation Of RNA Biosynthetic Process Positive Regulation Of Biosynthetic Process Protein-containing Complex Positive Regulation Of Macromolecule Metabolic Process Positive Regulation Of Metabolic Process MRNA Binding Protein-containing Complex Assembly Ribonucleoprotein Complex Regulation Of Macromolecule Metabolic Process MRNA Splicing, Via Spliceosome Negative Regulation Of Nucleobase-containing Compound Metabolic Process Regulation Of Primary Metabolic Process Negative Regulation Of Macromolecule Biosynthetic Process Negative Regulation Of RNA Metabolic Process Protein-containing Complex Organization Negative Regulation Of Biosynthetic Process Negative Regulation Of Macromolecule Metabolic Process Catalytic Step 2 Spliceosome Negative Regulation Of DNA-templated Transcription Negative Regulation Of RNA Biosynthetic Process Cytoplasm Regulation Of Metabolic Process Regulation Of Transcription By RNA Polymerase II Positive Regulation Of Nucleobase-containing Compound Metabolic Process