Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Matrin-3

Gene

MATR3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

May play a role in transcription or may interact with other nuclear matrix proteins to form the internal fibrogranular network. In association with the SFPQ-NONO heteromer may play a role in nuclear retention of defective RNAs.1 Publication

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri801 – 832Matrin-typePROSITE-ProRule annotationAdd BLAST32

GO - Molecular functioni

  • nucleotide binding Source: InterPro
  • poly(A) RNA binding Source: UniProtKB
  • structural molecule activity Source: ProtInc
  • zinc ion binding Source: InterPro

GO - Biological processi

  • posttranscriptional regulation of gene expression Source: CACAO
Complete GO annotation...

Keywords - Ligandi

Metal-binding, RNA-binding, Zinc

Enzyme and pathway databases

BioCyciZFISH:ENSG00000015479-MONOMER.

Names & Taxonomyi

Protein namesi
Recommended name:
Matrin-3
Gene namesi
Name:MATR3
Synonyms:KIAA0723
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 5

Organism-specific databases

HGNCiHGNC:6912. MATR3.

Subcellular locationi

GO - Cellular componenti

  • membrane Source: UniProtKB
  • nuclear inner membrane Source: ProtInc
  • nuclear matrix Source: UniProtKB-SubCell
  • nucleoplasm Source: HPA
Complete GO annotation...

Keywords - Cellular componenti

Nucleus

Pathology & Biotechi

Involvement in diseasei

Amyotrophic lateral sclerosis 21 (ALS21)3 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA neurodegenerative disorder affecting upper and lower motor neurons, resulting in muscle weakness and respiratory failure. Some patients may develop myopathic features or dementia.
See also OMIM:606070
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07406772A → T in ALS21; unknown pathological significance. 1 Publication1
Natural variantiVAR_06342185S → C in ALS21; results in increased interaction with TARDBP. 2 PublicationsCorresponds to variant rs121434591dbSNPEnsembl.1
Natural variantiVAR_071078115F → C in ALS21. 1 PublicationCorresponds to variant rs587777300dbSNPEnsembl.1
Natural variantiVAR_071079154P → S in ALS21; unknown pathological significance. 1 PublicationCorresponds to variant rs587777302dbSNPEnsembl.1
Natural variantiVAR_071080622T → A in ALS21. 1 PublicationCorresponds to variant rs587777301dbSNPEnsembl.1

Keywords - Diseasei

Amyotrophic lateral sclerosis, Disease mutation, Neurodegeneration

Organism-specific databases

DisGeNETi9782.
MalaCardsiMATR3.
MIMi606070. phenotype.
OpenTargetsiENSG00000015479.
ENSG00000280987.
Orphaneti803. Amyotrophic lateral sclerosis.
600. Distal myopathy with vocal cord weakness.
PharmGKBiPA30655.

Polymorphism and mutation databases

BioMutaiMATR3.
DMDMi12643409.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources1 Publication
ChainiPRO_00000816222 – 847Matrin-3Add BLAST846

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineCombined sources1 Publication1
Modified residuei3N6-acetyllysine; alternateCombined sources1
Cross-linki3Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateCombined sources
Modified residuei4PhosphoserineCombined sources1
Modified residuei9PhosphoserineCombined sources1
Modified residuei11PhosphoserineCombined sources1
Modified residuei14PhosphoserineCombined sources1
Modified residuei22PhosphoserineCombined sources1
Modified residuei41PhosphoserineCombined sources1
Modified residuei118PhosphoserineCombined sources1
Modified residuei126PhosphoserineCombined sources1
Cross-linki146Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei150PhosphothreonineCombined sources1
Modified residuei157PhosphoserineCombined sources1
Modified residuei158PhosphotyrosineCombined sources1
Modified residuei164PhosphoserineCombined sources1
Modified residuei188PhosphoserineCombined sources1
Modified residuei195PhosphoserineCombined sources1
Modified residuei202PhosphotyrosineCombined sources1
Modified residuei206PhosphoserineCombined sources1
Modified residuei208PhosphoserineCombined sources1
Modified residuei211PhosphoserineCombined sources1
Modified residuei219PhosphotyrosineCombined sources1
Modified residuei234PhosphoserineCombined sources1
Modified residuei264PhosphoserineCombined sources1
Modified residuei275PhosphoserineCombined sources1
Cross-linki487Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Cross-linki491Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei509PhosphoserineCombined sources1
Modified residuei511PhosphoserineCombined sources1
Modified residuei522N6-acetyllysineCombined sources1
Modified residuei533PhosphoserineCombined sources1
Cross-linki554Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei571N6-acetyllysineCombined sources1
Modified residuei596PhosphoserineCombined sources1
Modified residuei598PhosphoserineCombined sources1
Modified residuei604PhosphoserineCombined sources1
Modified residuei606PhosphoserineCombined sources1
Cross-linki617Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei654PhosphoserineCombined sources1
Modified residuei671PhosphoserineCombined sources1
Modified residuei673PhosphoserineCombined sources1
Modified residuei674PhosphoserineCombined sources1
Modified residuei679PhosphothreonineCombined sources1
Modified residuei689PhosphoserineCombined sources1
Cross-linki719Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Cross-linki736Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources
Modified residuei741PhosphothreonineCombined sources1
Modified residuei747PhosphoserineCombined sources1
Modified residuei759PhosphoserineBy similarity1
Modified residuei766PhosphoserineCombined sources1
Modified residuei836N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation, Isopeptide bond, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiP43243.
MaxQBiP43243.
PaxDbiP43243.
PeptideAtlasiP43243.
PRIDEiP43243.
TopDownProteomicsiP43243-1. [P43243-1]

PTM databases

iPTMnetiP43243.
PhosphoSitePlusiP43243.
SwissPalmiP43243.

Miscellaneous databases

PMAP-CutDBP43243.

Expressioni

Gene expression databases

BgeeiENSG00000015479.
CleanExiHS_MATR3.
ExpressionAtlasiP43243. baseline and differential.
GenevisibleiP43243. HS.

Organism-specific databases

HPAiCAB033552.
HPA036564.
HPA036565.

Interactioni

Subunit structurei

Part of a complex consisting of SFPQ, NONO and MATR3. Interacts with AGO1 and AGO2. Part of a complex composed at least of ASCL2, EMSY, HCFC1, HSPA8, CCAR2, MATR3, MKI67, RBBP5, TUBB2A, WDR5 and ZNF335; this complex may have a histone H3-specific methyltransferase activity. Interacts with TARDBP.4 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
HNRPKQ6IBN13EBI-352602,EBI-3440248
NR4A1P227362EBI-352602,EBI-721550
RASD1Q9Y2723EBI-352602,EBI-740818

Protein-protein interaction databases

BioGridi115126. 188 interactors.
IntActiP43243. 146 interactors.
MINTiMINT-4999417.
STRINGi9606.ENSP00000354346.

Structurei

3D structure databases

ProteinModelPortaliP43243.
SMRiP43243.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini398 – 473RRM 1PROSITE-ProRule annotationAdd BLAST76
Domaini496 – 571RRM 2PROSITE-ProRule annotationAdd BLAST76

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi710 – 718Nuclear localization signalSequence analysis9

Sequence similaritiesi

Contains 1 matrin-type zinc finger.PROSITE-ProRule annotation
Contains 2 RRM (RNA recognition motif) domains.PROSITE-ProRule annotation

Zinc finger

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Zinc fingeri801 – 832Matrin-typePROSITE-ProRule annotationAdd BLAST32

Keywords - Domaini

Repeat, Zinc-finger

Phylogenomic databases

eggNOGiENOG410IGPC. Eukaryota.
ENOG410XSPB. LUCA.
GeneTreeiENSGT00850000132320.
HOGENOMiHOG000015369.
HOVERGENiHBG057347.
InParanoidiP43243.
KOiK13213.
OrthoDBiEOG091G0QCV.
PhylomeDBiP43243.
TreeFamiTF333921.

Family and domain databases

Gene3Di3.30.70.330. 2 hits.
InterProiIPR012677. Nucleotide-bd_a/b_plait.
IPR000504. RRM_dom.
IPR000690. Znf_C2H2_matrin.
IPR003604. Znf_U1.
[Graphical view]
SMARTiSM00360. RRM. 2 hits.
SM00451. ZnF_U1. 2 hits.
[Graphical view]
SUPFAMiSSF54928. SSF54928. 2 hits.
PROSITEiPS50102. RRM. 2 hits.
PS50171. ZF_MATRIN. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P43243-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MSKSFQQSSL SRDSQGHGRD LSAAGIGLLA AATQSLSMPA SLGRMNQGTA
60 70 80 90 100
RLASLMNLGM SSSLNQQGAH SALSSASTSS HNLQSIFNIG SRGPLPLSSQ
110 120 130 140 150
HRGDADQASN ILASFGLSAR DLDELSRYPE DKITPENLPQ ILLQLKRRRT
160 170 180 190 200
EEGPTLSYGR DGRSATREPP YRVPRDDWEE KRHFRRDSFD DRGPSLNPVL
210 220 230 240 250
DYDHGSRSQE SGYYDRMDYE DDRLRDGERC RDDSFFGETS HNYHKFDSEY
260 270 280 290 300
ERMGRGPGPL QERSLFEKKR GAPPSSNIED FHGLLPKGYP HLCSICDLPV
310 320 330 340 350
HSNKEWSQHI NGASHSRRCQ LLLEIYPEWN PDNDTGHTMG DPFMLQQSTN
360 370 380 390 400
PAPGILGPPP PSFHLGGPAV GPRGNLGAGN GNLQGPRHMQ KGRVETSRVV
410 420 430 440 450
HIMDFQRGKN LRYQLLQLVE PFGVISNHLI LNKINEAFIE MATTEDAQAA
460 470 480 490 500
VDYYTTTPAL VFGKPVRVHL SQKYKRIKKP EGKPDQKFDQ KQELGRVIHL
510 520 530 540 550
SNLPHSGYSD SAVLKLAEPY GKIKNYILMR MKSQAFIEME TREDAMAMVD
560 570 580 590 600
HCLKKALWFQ GRCVKVDLSE KYKKLVLRIP NRGIDLLKKD KSRKRSYSPD
610 620 630 640 650
GKESPSDKKS KTDGSQKTES STEGKEQEEK SGEDGEKDTK DDQTEQEPNM
660 670 680 690 700
LLESEDELLV DEEEAAALLE SGSSVGDETD LANLGDVASD GKKEPSDKAV
710 720 730 740 750
KKDGSASAAA KKKLKKVDKI EELDQENEAA LENGIKNEEN TEPGAESSEN
760 770 780 790 800
ADDPNKDTSE NADGQSDENK DDYTIPDEYR IGPYQPNVPV GIDYVIPKTG
810 820 830 840
FYCKLCSLFY TNEEVAKNTH CSSLPHYQKL KKFLNKLAEE RRQKKET
Length:847
Mass (Da):94,623
Last modified:January 24, 2001 - v2
Checksum:i530AA49214BC1611
GO
Isoform 2 (identifier: P43243-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-304: MSKSFQQSSL...ICDLPVHSNK → MLGAQWRRNQPSRAAE

Note: No experimental confirmation available.
Show »
Length:559
Mass (Da):62,576
Checksum:i7E7772B22A7AF741
GO

Sequence cautioni

The sequence AAF17217 differs from that shown. Reason: Frameshift at several positions.Curated
The sequence BAA34443 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti257P → S in AAF17217 (PubMed:10931946).Curated1
Sequence conflicti274P → S in AAF17217 (PubMed:10931946).Curated1
Sequence conflicti572Y → C in AAF17217 (PubMed:10931946).Curated1
Sequence conflicti572Y → C in M63483 (PubMed:2033075).Curated1
Sequence conflicti691G → P in M63483 (PubMed:2033075).Curated1
Sequence conflicti703D → H in M63483 (PubMed:2033075).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07406772A → T in ALS21; unknown pathological significance. 1 Publication1
Natural variantiVAR_06342185S → C in ALS21; results in increased interaction with TARDBP. 2 PublicationsCorresponds to variant rs121434591dbSNPEnsembl.1
Natural variantiVAR_07406889I → V.1 PublicationCorresponds to variant rs528548235dbSNPEnsembl.1
Natural variantiVAR_071078115F → C in ALS21. 1 PublicationCorresponds to variant rs587777300dbSNPEnsembl.1
Natural variantiVAR_071079154P → S in ALS21; unknown pathological significance. 1 PublicationCorresponds to variant rs587777302dbSNPEnsembl.1
Natural variantiVAR_071080622T → A in ALS21. 1 PublicationCorresponds to variant rs587777301dbSNPEnsembl.1
Natural variantiVAR_074069664E → A.1 PublicationCorresponds to variant rs139589527dbSNPEnsembl.1
Natural variantiVAR_074070787N → S.1 PublicationCorresponds to variant rs148402819dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0426241 – 304MSKSF…VHSNK → MLGAQWRRNQPSRAAE in isoform 2. 1 PublicationAdd BLAST304

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB018266 mRNA. Translation: BAA34443.2. Different initiation.
AF117236 mRNA. Translation: AAF17217.1. Frameshift.
AK316420 mRNA. Translation: BAH14791.1.
AC011404 Genomic DNA. No translation available.
CH471062 Genomic DNA. Translation: EAW62114.1.
CH471062 Genomic DNA. Translation: EAW62115.1.
CH471062 Genomic DNA. Translation: EAW62116.1.
CH471062 Genomic DNA. Translation: EAW62117.1.
BC015031 mRNA. Translation: AAH15031.1.
M63483 mRNA. No translation available.
CCDSiCCDS4210.1. [P43243-1]
CCDS54908.1. [P43243-2]
RefSeqiNP_001181883.1. NM_001194954.1. [P43243-1]
NP_001181884.1. NM_001194955.1. [P43243-1]
NP_001181885.1. NM_001194956.1. [P43243-2]
NP_001269207.1. NM_001282278.1.
NP_061322.2. NM_018834.5. [P43243-1]
NP_954659.1. NM_199189.2. [P43243-1]
UniGeneiHs.268939.
Hs.595110.

Genome annotation databases

EnsembliENST00000394805; ENSP00000378284; ENSG00000015479. [P43243-1]
ENST00000503811; ENSP00000423587; ENSG00000015479. [P43243-2]
GeneIDi9782.
KEGGihsa:9782.
UCSCiuc003ldx.4. human. [P43243-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB018266 mRNA. Translation: BAA34443.2. Different initiation.
AF117236 mRNA. Translation: AAF17217.1. Frameshift.
AK316420 mRNA. Translation: BAH14791.1.
AC011404 Genomic DNA. No translation available.
CH471062 Genomic DNA. Translation: EAW62114.1.
CH471062 Genomic DNA. Translation: EAW62115.1.
CH471062 Genomic DNA. Translation: EAW62116.1.
CH471062 Genomic DNA. Translation: EAW62117.1.
BC015031 mRNA. Translation: AAH15031.1.
M63483 mRNA. No translation available.
CCDSiCCDS4210.1. [P43243-1]
CCDS54908.1. [P43243-2]
RefSeqiNP_001181883.1. NM_001194954.1. [P43243-1]
NP_001181884.1. NM_001194955.1. [P43243-1]
NP_001181885.1. NM_001194956.1. [P43243-2]
NP_001269207.1. NM_001282278.1.
NP_061322.2. NM_018834.5. [P43243-1]
NP_954659.1. NM_199189.2. [P43243-1]
UniGeneiHs.268939.
Hs.595110.

3D structure databases

ProteinModelPortaliP43243.
SMRiP43243.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115126. 188 interactors.
IntActiP43243. 146 interactors.
MINTiMINT-4999417.
STRINGi9606.ENSP00000354346.

PTM databases

iPTMnetiP43243.
PhosphoSitePlusiP43243.
SwissPalmiP43243.

Polymorphism and mutation databases

BioMutaiMATR3.
DMDMi12643409.

Proteomic databases

EPDiP43243.
MaxQBiP43243.
PaxDbiP43243.
PeptideAtlasiP43243.
PRIDEiP43243.
TopDownProteomicsiP43243-1. [P43243-1]

Protocols and materials databases

DNASUi9782.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000394805; ENSP00000378284; ENSG00000015479. [P43243-1]
ENST00000503811; ENSP00000423587; ENSG00000015479. [P43243-2]
GeneIDi9782.
KEGGihsa:9782.
UCSCiuc003ldx.4. human. [P43243-1]

Organism-specific databases

CTDi9782.
DisGeNETi9782.
GeneCardsiMATR3.
HGNCiHGNC:6912. MATR3.
HPAiCAB033552.
HPA036564.
HPA036565.
MalaCardsiMATR3.
MIMi164015. gene.
606070. phenotype.
neXtProtiNX_P43243.
OpenTargetsiENSG00000015479.
ENSG00000280987.
Orphaneti803. Amyotrophic lateral sclerosis.
600. Distal myopathy with vocal cord weakness.
PharmGKBiPA30655.
HUGEiSearch...
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IGPC. Eukaryota.
ENOG410XSPB. LUCA.
GeneTreeiENSGT00850000132320.
HOGENOMiHOG000015369.
HOVERGENiHBG057347.
InParanoidiP43243.
KOiK13213.
OrthoDBiEOG091G0QCV.
PhylomeDBiP43243.
TreeFamiTF333921.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000015479-MONOMER.

Miscellaneous databases

ChiTaRSiMATR3. human.
GeneWikiiMATR3.
GenomeRNAii9782.
PMAP-CutDBP43243.
PROiP43243.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000015479.
CleanExiHS_MATR3.
ExpressionAtlasiP43243. baseline and differential.
GenevisibleiP43243. HS.

Family and domain databases

Gene3Di3.30.70.330. 2 hits.
InterProiIPR012677. Nucleotide-bd_a/b_plait.
IPR000504. RRM_dom.
IPR000690. Znf_C2H2_matrin.
IPR003604. Znf_U1.
[Graphical view]
SMARTiSM00360. RRM. 2 hits.
SM00451. ZnF_U1. 2 hits.
[Graphical view]
SUPFAMiSSF54928. SSF54928. 2 hits.
PROSITEiPS50102. RRM. 2 hits.
PS50171. ZF_MATRIN. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMATR3_HUMAN
AccessioniPrimary (citable) accession number: P43243
Secondary accession number(s): B7ZAV5
, D3DQC3, Q9UHW0, Q9UQ27
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1995
Last sequence update: January 24, 2001
Last modified: November 30, 2016
This is version 182 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 5
    Human chromosome 5: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.