ID:SMAD3_HUMAN DESCRIPTION: RecName: Full=Mothers against decapentaplegic homolog 3; Short=MAD homolog 3; Short=Mad3; Short=Mothers against DPP homolog 3; Short=hMAD-3; AltName: Full=JV15-2; AltName: Full=SMAD family member 3; Short=SMAD 3; Short=Smad3; Short=hSMAD3; FUNCTION: Receptor-regulated SMAD (R-SMAD) that is an intracellular signal transducer and transcriptional modulator activated by TGF-beta (transforming growth factor) and activin type 1 receptor kinases. Binds the TRE element in the promoter region of many genes that are regulated by TGF-beta and, on formation of the SMAD3/SMAD4 complex, activates transcription. Also can form a SMAD3/SMAD4/JUN/FOS complex at the AP-1/SMAD site to regulate TGF-beta-mediated transcription. Has an inhibitory effect on wound healing probably by modulating both growth and migration of primary keratinocytes and by altering the TGF- mediated chemotaxis of monocytes. This effect on wound healing appears to be hormone-sensitive. Regulator of chondrogenesis and osteogenesis and inhibits early healing of bone fractures (By similarity). Positively regulates PDPK1 kinase activity by stimulating its dissociation from the 14-3-3 protein YWHAQ which acts as a negative regulator. SUBUNIT: Monomer; in the absence of TGF-beta. Homooligomer; in the presence of TGF-beta. Heterotrimer; forms a heterotrimer in the presence of TGF-beta consisting of two molecules of C-terminally phosphorylated SMAD2 or SMAD3 and one of SMAD4 to form the transcriptionally active SMAD2/SMAD3-SMAD4 complex. Interacts with TGFBR1. Part of a complex consisting of AIP1, ACVR2A, ACVR1B and SMAD3. Interacts with AIP1, TGFB1I1, TTRAP, FOXL2, PML, PRDM16, HGS and WWP1. Interacts (via MH2 domain) with CITED2 (via C- terminus) (By similarity). Interacts with NEDD4L; the interaction requires TGF-beta stimulation (By similarity). Interacts (via the MH2 domain) with ZFYVE9. Interacts with HDAC1, VDR, TGIF and TGIF2, RUNX3, CREBBP, SKOR1, SKOR2, SNON, ATF2, SMURF2 and TGFB1I1. Interacts with DACH1; the interaction inhibits the TGF- beta signaling. Forms a complex with SMAD2 and TRIM33 upon addition of TGF-beta. Found in a complex with SMAD3, RAN and XPO4. Interacts in the complex directly with XPO4. Interacts (via the MH2 domain) with LEMD3; the interaction represses SMAD3 transcriptional activity through preventing the formation of the heteromeric complex with SMAD4 and translocation to the nucleus. Interacts with RBPMS. Interacts (via MH2 domain) with MECOM. Interacts with WWTR1 (via its coiled-coil domain). Interacts (via the linker region) with EP300 (C-terminal); the interaction promotes SMAD3 acetylation and is enhanced by TGF-beta phosphorylation in the C-terminal of SMAD3. This interaction can be blocked by competitive binding of adenovirus oncoprotein E1A to the same C-terminal site on EP300, which then results in partially inhibited SMAD3/SMAD4 transcriptional activity. Interacts with SKI; the interaction represses SMAD3 transcriptional activity. Component of the multimeric complex SMAD3/SMAD4/JUN/FOS which forms at the AP1 promoter site; required for syngernistic transcriptional activity in response to TGF-beta. Interacts (via an N-terminal domain) with JUN (via its basic DNA binding and leucine zipper domains); this interaction is essential for DNA binding and cooperative transcriptional activity in response to TGF-beta. Interacts with PPM1A; the interaction dephosphorylates SMAD3 in the C-terminal SXS motif leading to disruption of the SMAD2/3-SMAD4 complex, nuclear export and termination of TGF-beta signaling. Interacts (dephosphorylated form via the MH1 and MH2 domains) with RANBP3 (via its C-terminal R domain); the interaction results in the export of dephosphorylated SMAD3 out of the nucleus and termination of the TGF-beta signaling. Interacts with MEN1. Interacts with IL1F7. Interaction with CSNK1G2. Interacts with PDPK1 (via PH domain). Interacts with DAB2; the interactions are enhanced upon TGF-beta stimulation. Interacts with USP15. INTERACTION: P60709:ACTB; NbExp=3; IntAct=EBI-347161, EBI-353944; Q9H2X0:CHRD; NbExp=2; IntAct=EBI-347161, EBI-947551; P98082:DAB2; NbExp=3; IntAct=EBI-347161, EBI-1171238; Q9BZ29:DOCK9; NbExp=3; IntAct=EBI-347161, EBI-2695893; Q99836:MYD88; NbExp=3; IntAct=EBI-347161, EBI-447677; Q16822:PCK2; NbExp=2; IntAct=EBI-347161, EBI-2825219; Q9BZL4:PPP1R12C; NbExp=2; IntAct=EBI-347161, EBI-721802; P24158:PRTN3; NbExp=2; IntAct=EBI-347161, EBI-465028; Q96EP0:RNF31; NbExp=2; IntAct=EBI-347161, EBI-948111; Q9BYW2:SETD2; NbExp=2; IntAct=EBI-347161, EBI-945869; Q15796:SMAD2; NbExp=2; IntAct=EBI-347161, EBI-1040141; Q13485:SMAD4; NbExp=9; IntAct=EBI-347161, EBI-347263; Q13501:SQSTM1; NbExp=3; IntAct=EBI-347161, EBI-307104; Q12772:SREBF2; NbExp=3; IntAct=EBI-347161, EBI-465059; Q05066:SRY; NbExp=3; IntAct=EBI-347161, EBI-464987; Q9Y3Q8:TSC22D4; NbExp=2; IntAct=EBI-347161, EBI-739485; Q93009:USP7; NbExp=2; IntAct=EBI-347161, EBI-302474; O00308:WWP2; NbExp=4; IntAct=EBI-347161, EBI-743923; Q5D1E8:ZC3H12A; NbExp=2; IntAct=EBI-347161, EBI-747793; SUBCELLULAR LOCATION: Cytoplasm. Nucleus. Note=Cytoplasmic and nuclear in the absence of TGF-beta. On TGF-beta stimulation, migrates to the nucleus when complexed with SMAD4. Through the action of the phosphatase PPM1A, released from the SMAD2/SMAD4 complex, and exported out of the nucleus by interaction with RANBP1. Co-localizes with LEMD3 at the nucleus inner membrane. MAPK-mediated phosphorylation appears to have no effect on nuclear import. PDPK1 prevents its nuclear translocation in response to TGF-beta. DOMAIN: The MH1 domain is required for DNA binding. Also binds zinc ions which are necessary for the DNA binding. DOMAIN: The MH2 domain is required for both homomeric and heteromeric interactions and for transcriptional regulation. Sufficient for nuclear import. DOMAIN: The linker region is required for the TGFbeta-mediated transcriptional activity and acts synergistically with the MH2 domain. PTM: Phosphorylated on serine and threonine residues. Enhanced phosphorylation in the linker region on Thr-179, Ser-204 and Ser- 208 on EGF AND TGF-beta treatment. Ser-208 is the main site of MAPK-mediated phosphorylation. CDK-mediated phosphorylation occurs in a cell-cycle dependent manner and inhibits both the transcriptional activity and antiproliferative functions of SMAD3. This phosphorylation is inhibited by flavopiridol. Maximum phosphorylation at the G(1)/S junction. Also phosphorylated on serine residues in the C-terminal SXS motif by TGFBR1 and ACVR1. TGFBR1-mediated phosphorylation at these C-terminal sites is required for interaction with SMAD4, nuclear location and transactivational activity, and appears to be a prerequisite for the TGF-beta mediated phosphorylation in the linker region. Dephosphorylated in the C-terminal SXS motif by PPM1A. This dephosphorylation disrupts the interaction with SMAD4, promotes nuclear export and terminates TGF-beta-mediated signaling. Phosphorylation at Ser-418 by CSNK1G2/CK1 promotes ligand- dependent ubiquitination and subsequent proteasome degradation, thus inhibiting SMAD3-mediated TGF-beta responses. Phosphorylated by PDPK1. PTM: Acetylation in the nucleus by EP300 in the MH2 domain regulates positively its transcriptional activity and is enhanced by TGF-beta. PTM: Ubiquitinated. Monoubiquitinated, leading to prevent DNA- binding. Deubiquitination by USP15 alleviates inhibition and promotes activation of TGF-beta target genes. DISEASE: Defects in SMAD3 may be a cause of colorectal cancer (CRC) [MIM:114500]. DISEASE: Defects in SMAD3 are the cause of Loeys-Dietz syndrome 3 (LDS3) [MIM:613795]. An aortic aneurysm syndrome with widespread systemic involvement. The disorder is characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula or cleft palate. Patients with LDS3 also manifest early- onset osteoarthritis. They lack craniosynostosis and mental retardation. Note=SMAD3 mutations have been reported to be also associated with thoracic aortic aneurysms and dissection (TAAD) (PubMed:21778426). This phenotype is distinguised from LDS3 by having aneurysms restricted to thoracic aorta. As individuals carrying these mutations also exhibit aneurysms of other arteries, including abdominal aorta, iliac, and/or intracranial arteries (PubMed:21778426), they have been classified as LDS3 by the OMIM resource. SIMILARITY: Belongs to the dwarfin/SMAD family. SIMILARITY: Contains 1 MH1 (MAD homology 1) domain. SIMILARITY: Contains 1 MH2 (MAD homology 2) domain.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P84022
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
AK290881 - Homo sapiens cDNA FLJ76792 complete cds, highly similar to Homo sapiens SMAD, mothers against DPP homolog 3 (Drosophila) (SMAD3), mRNA. HW061230 - JP 2012529430-A/105: METHODS FOR TREATING CHRONIC KIDNEY DISEASE. HW286095 - JP 2013143917-A/1: CURATIVE OR PREVENTIVE AGENT FOR FIBROSIS. JB252038 - Sequence 105 from Patent EP2440214. LP764937 - Sequence 105 from Patent EP3276004. LX148400 - JP 2017079741-A/1: CURATIVE OR PREVENTIVE AGENT FOR FIBROSIS. BC050743 - Homo sapiens SMAD family member 3, mRNA (cDNA clone MGC:60396 IMAGE:6094328), complete cds. U68019 - Homo sapiens mad protein homolog (hMAD-3) mRNA, complete cds. U76622 - Human mad homolog JV15-2 mRNA, complete cds. AB464213 - Synthetic construct DNA, clone: pF1KB8191, Homo sapiens SMAD3 gene for SMAD family member 3, without stop codon, in Flexi system. EU446487 - Synthetic construct Homo sapiens clone IMAGE:100069864; IMAGE:100011696; FLH258539.01L SMAD family member 3 (SMAD3) gene, encodes complete protein. KJ897155 - Synthetic construct Homo sapiens clone ccsbBroadEn_06549 SMAD3 gene, encodes complete protein. KR711077 - Synthetic construct Homo sapiens clone CCSBHm_00020082 SMAD3 (SMAD3) mRNA, encodes complete protein. KR712265 - Synthetic construct Homo sapiens clone CCSBHm_00900229 SMAD3 (SMAD3) mRNA, encodes complete protein. AB209616 - Homo sapiens mRNA for MAD, mothers against decapentaplegic homolog 3 variant protein. JD396566 - Sequence 377590 from Patent EP1572962. JD383492 - Sequence 364516 from Patent EP1572962. JD452263 - Sequence 433287 from Patent EP1572962. JD387343 - Sequence 368367 from Patent EP1572962. JD456211 - Sequence 437235 from Patent EP1572962. HW061231 - JP 2012529430-A/106: METHODS FOR TREATING CHRONIC KIDNEY DISEASE. JB252039 - Sequence 106 from Patent EP2440214. LP764938 - Sequence 106 from Patent EP3276004. AK316017 - Homo sapiens cDNA, FLJ78916 complete cds, highly similar to Mothers against decapentaplegic homolog 3 (SMAD3). AK298139 - Homo sapiens cDNA FLJ54532 complete cds, highly similar to Mothers against decapentaplegic homolog 3 (SMAD3). HW061232 - JP 2012529430-A/107: METHODS FOR TREATING CHRONIC KIDNEY DISEASE. JB252040 - Sequence 107 from Patent EP2440214. LP764939 - Sequence 107 from Patent EP3276004. EU016553 - Homo sapiens mutant mothers against decapentaplegic 3 (SMAD3) mRNA, partial sequence; alternatively spliced. HW061233 - JP 2012529430-A/108: METHODS FOR TREATING CHRONIC KIDNEY DISEASE. JB252041 - Sequence 108 from Patent EP2440214. LP764940 - Sequence 108 from Patent EP3276004. AK300614 - Homo sapiens cDNA FLJ59989 complete cds, highly similar to Mothers against decapentaplegic homolog 3 (SMAD3). AK301348 - Homo sapiens cDNA FLJ56254 complete cds, highly similar to Mothers against decapentaplegic homolog 3 (SMAD3). AK026690 - Homo sapiens cDNA: FLJ23037 fis, clone LNG02036, highly similar to HSU68019 Homo sapiens mad protein homolog (hMAD-3) mRNA. EU016554 - Homo sapiens mutant mothers against decapentaplegic 3 (SMAD3) mRNA, partial sequence; alternatively spliced. EU016552 - Homo sapiens mutant mothers against decapentaplegic 3 (SMAD3) mRNA, partial sequence; alternatively spliced. CR749287 - Homo sapiens mRNA; cDNA DKFZp686J10186 (from clone DKFZp686J10186). JD350828 - Sequence 331852 from Patent EP1572962. JD475131 - Sequence 456155 from Patent EP1572962. JD188508 - Sequence 169532 from Patent EP1572962. JD557713 - Sequence 538737 from Patent EP1572962. JD482782 - Sequence 463806 from Patent EP1572962. JD153362 - Sequence 134386 from Patent EP1572962. JD436334 - Sequence 417358 from Patent EP1572962. JD191218 - Sequence 172242 from Patent EP1572962. JD390692 - Sequence 371716 from Patent EP1572962. JD184078 - Sequence 165102 from Patent EP1572962. JD067622 - Sequence 48646 from Patent EP1572962. JD091786 - Sequence 72810 from Patent EP1572962. JD439431 - Sequence 420455 from Patent EP1572962. JD387163 - Sequence 368187 from Patent EP1572962. JD322573 - Sequence 303597 from Patent EP1572962. JD377118 - Sequence 358142 from Patent EP1572962. HV347492 - JP 2011513238-A/19: MICRO-RNAS THAT MODULATE SMOOTH MUSCLE PROLIFERATION AND DIFFERENTIATION AND USES THEREOF. DQ579122 - Homo sapiens piRNA piR-47234, complete sequence. AL110265 - Homo sapiens mRNA; cDNA DKFZp586N0721 (from clone DKFZp586N0721). JD343511 - Sequence 324535 from Patent EP1572962. JD036755 - Sequence 17779 from Patent EP1572962. JD546145 - Sequence 527169 from Patent EP1572962. JD098131 - Sequence 79155 from Patent EP1572962. JD067771 - Sequence 48795 from Patent EP1572962. JD403791 - Sequence 384815 from Patent EP1572962. JD555622 - Sequence 536646 from Patent EP1572962. JD416084 - Sequence 397108 from Patent EP1572962. JD061538 - Sequence 42562 from Patent EP1572962. AK313812 - Homo sapiens cDNA, FLJ94430. JD196988 - Sequence 178012 from Patent EP1572962. JD387579 - Sequence 368603 from Patent EP1572962. JD234604 - Sequence 215628 from Patent EP1572962. AF151027 - Homo sapiens HSPC193 mRNA, complete cds. BC000414 - Homo sapiens SMAD family member 3, mRNA (cDNA clone IMAGE:2961412). BC007496 - Homo sapiens SMAD family member 3, mRNA (cDNA clone IMAGE:2961412). KJ901561 - Synthetic construct Homo sapiens clone ccsbBroadEn_10955 SMAD3 gene, encodes complete protein. CR457386 - Homo sapiens full open reading frame cDNA clone RZPDo834F0510D for gene DKFZP586N0721, DKFZP586N0721 protein; complete cds, incl. stopcodon. JD513835 - Sequence 494859 from Patent EP1572962. JD449244 - Sequence 430268 from Patent EP1572962. JD352725 - Sequence 333749 from Patent EP1572962. JD122805 - Sequence 103829 from Patent EP1572962. JD089916 - Sequence 70940 from Patent EP1572962. JD350519 - Sequence 331543 from Patent EP1572962. JD284540 - Sequence 265564 from Patent EP1572962. JD228736 - Sequence 209760 from Patent EP1572962. JD104195 - Sequence 85219 from Patent EP1572962. JD290532 - Sequence 271556 from Patent EP1572962. JD154802 - Sequence 135826 from Patent EP1572962. JD205715 - Sequence 186739 from Patent EP1572962. JD325365 - Sequence 306389 from Patent EP1572962. JD526534 - Sequence 507558 from Patent EP1572962.
Biochemical and Signaling Pathways
BioCarta from NCI Cancer Genome Anatomy Project h_g1Pathway - Cell Cycle: G1/S Check Point h_nthiPathway - NFkB activation by Nontypeable Hemophilus influenzae h_tgfbPathway - TGF beta signaling pathway h_tob1Pathway - Role of Tob in T-cell activation
Reactome (by CSHL, EBI, and GO)
Protein P84022 (Reactome details) participates in the following event(s):
R-HSA-2187375 SMAD3 binds STUB1 (CHIP) R-HSA-9008692 IL37(?-218) binds SMAD3 R-HSA-9009910 IL37(?-218) binds p-S423,S425-SMAD3 R-HSA-2187368 STUB1 (CHIP) ubiquitinates SMAD3 R-HSA-170835 An anchoring protein, ZFYVE9 (SARA), recruits SMAD2/3 R-HSA-1181355 Phosphorylation of R-SMAD2/3 by NODAL receptor R-HSA-1549526 Phosphorylation of SMAD2/3 by Activin:Activin Receptor R-HSA-2187358 PMEPA1 sequesters unphosphorylated SMAD2/3 R-HSA-2187395 Dephosphorylated SMAD2/3 translocates to the cytosol R-HSA-2187401 MTMR4 dephosphorylates SMAD2/3 R-HSA-170847 Phosphorylated SMAD2 and SMAD3 form a complex with SMAD4 R-HSA-2187355 PMEPA1 sequesters phosphorylated SMAD2/3 R-HSA-2187405 MTMR4 binds phosphorylated SMAD2/3 R-HSA-170850 Phosphorylated SMAD2/3 dissociates from TGFBR R-HSA-8878143 RUNX3 binds SMAD3 and SMAD4 R-HSA-870449 TRIM33 monoubiquitinates SMAD4 R-HSA-209055 PPM1A dephosphorylates nuclear SMAD2/3 R-HSA-170868 Activated type I receptor phosphorylates SMAD2/3 directly R-HSA-6781764 USP15 deubiquitinates SMAD1,SMAD2,SMAD3, SMAD7:SMURF,KEAP1 R-HSA-173488 The SMAD2/3:SMAD4 complex transfers to the nucleus R-HSA-2031355 WWTR1 binds SMAD2/3:SMAD4 heterotrimer R-HSA-8878178 The complex of RUNX3, SMAD3 and SMAD4 binds the CDKN1A gene promoter R-HSA-8952226 RUNX3 binds the BCL2L11 (BIM) gene R-HSA-173481 SKI/SKIL binds SMAD complex, suppressing TGF-beta signaling R-HSA-870538 TRIM33 (Ectodermin) binds SMAD heterotrimer in the nucleus R-HSA-1225919 Phospho R-SMAD(SMAD2/3):CO-SMAD(SMAD4):FOXH1 binds Activin Response Element R-HSA-1535903 Phospho R-SMAD(SMAD2/3):CO-SMAD(SMAD4):FOXO3 binds FoxO3a-binding elements R-HSA-2127257 SMAD2/3:SMAD4 heterotrimer forms a complex with RBL1, E2F4/5 and DP1/2 R-HSA-2176475 Phosphorylation of SMAD2 and SMAD3 linker regions by CDK8 or CDK9 R-HSA-2186607 TGIF recruits HDAC1 to SMAD2/3:SMAD4 heterotrimer R-HSA-2186643 MEN1 binds SMAD2/3:SMAD4 heterotrimer R-HSA-2187309 SMAD2/3:SMAD4 heterotrimer binds SP1 R-HSA-2187330 PARP1 binds SMAD2/3:SMAD4 heterotrimer R-HSA-2187388 PPM1A protein phosphatase binds phosphorylated SMAD2/3 R-HSA-2186747 Ubiquitination of SKI/SKIL by RNF111/SMURF2 R-NUL-2186755 Ubiquitination of SKI/SKIL by Rnf111 R-HSA-2176491 NEDD4L binds phosphorylated linker region of SMAD2/3 R-HSA-2179274 SMURF2 binds SMAD3 phosphorylated in the linker region R-HSA-2179276 SMURF2 monoubiquitinates SMAD3 R-HSA-2187325 PARP1 ADP-ribosylates SMAD3 and SMAD4 R-HSA-3713560 An anchoring protein, ZFYVE9 (SARA), recruits SMAD2/3 phosphorylation motif mutants R-HSA-3702186 Phosphorylated SMAD2/3 MH2 domain mutants dissociate from TGFBR R-HSA-3702153 An anchoring protein, ZFYVE9 (SARA), recruits SMAD2/3 MH2 domain mutants R-HSA-2106579 WWTR1:SMAD translocates to the nucleus R-NUL-2186736 Rnf111 binds SKI/SKIL in complex with SMAD2/3:SMAD4 upon TGF-beta stimulation R-HSA-2186741 SMAD2/3 activation induces binding of RNF111/SMURF2 to SKI/SKIL R-HSA-2176502 NEDD4L ubiquitinates SMAD2/3 R-HSA-3702184 Activated type I receptor phosphorylates SMAD2/3 MH2 domain mutants directly R-HSA-2173788 Downregulation of TGF-beta receptor signaling R-HSA-9008059 Interleukin-37 signaling R-HSA-2173789 TGF-beta receptor signaling activates SMADs R-HSA-1181150 Signaling by NODAL R-HSA-1502540 Signaling by Activin R-HSA-3656532 TGFBR1 KD Mutants in Cancer R-HSA-2173795 Downregulation of SMAD2/3:SMAD4 transcriptional activity R-HSA-446652 Interleukin-1 family signaling R-HSA-2173796 SMAD2/SMAD3:SMAD4 heterotrimer regulates transcription R-HSA-3311021 SMAD4 MH2 Domain Mutants in Cancer R-HSA-8941855 RUNX3 regulates CDKN1A transcription R-HSA-170834 Signaling by TGF-beta Receptor Complex R-HSA-1266738 Developmental Biology R-HSA-9006936 Signaling by TGF-beta family members R-HSA-3656534 Loss of Function of TGFBR1 in Cancer R-HSA-2173793 Transcriptional activity of SMAD2/SMAD3:SMAD4 heterotrimer R-HSA-5689880 Ub-specific processing proteases R-HSA-449147 Signaling by Interleukins R-HSA-3304347 Loss of Function of SMAD4 in Cancer R-HSA-8952158 RUNX3 regulates BCL2L11 (BIM) transcription R-HSA-8878159 Transcriptional regulation by RUNX3 R-HSA-3304356 SMAD2/3 Phosphorylation Motif Mutants in Cancer R-HSA-3315487 SMAD2/3 MH2 Domain Mutants in Cancer R-HSA-162582 Signal Transduction R-HSA-3304351 Signaling by TGF-beta Receptor Complex in Cancer R-HSA-212436 Generic Transcription Pathway R-HSA-5688426 Deubiquitination R-HSA-1280215 Cytokine Signaling in Immune system R-HSA-3304349 Loss of Function of SMAD2/3 in Cancer R-HSA-5663202 Diseases of signal transduction R-HSA-73857 RNA Polymerase II Transcription R-HSA-597592 Post-translational protein modification R-HSA-168256 Immune System R-HSA-1643685 Disease R-HSA-74160 Gene expression (Transcription) R-HSA-392499 Metabolism of proteins