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Protein

G-rich sequence factor 1

Gene

GRSF1

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

Functioni

Regulator of post-transcriptional mitochondrial gene expression, required for assembly of the mitochondrial ribosome and for recruitment of mRNA and lncRNA. Binds RNAs containing the 14 base G-rich element. Preferentially binds RNAs transcribed from three contiguous genes on the light strand of mtDNA, the ND6 mRNA, and the long non-coding RNAs for MT-CYB and MT-ND5, each of which contains multiple consensus binding sequences.2 Publications

GO - Molecular functioni

  • mRNA binding Source: ProtInc
  • nucleotide binding Source: InterPro
  • poly(A) RNA binding Source: UniProtKB
  • RNA binding Source: ProtInc

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

mRNA processing, tRNA processing

Keywords - Ligandi

RNA-binding

Enzyme and pathway databases

BioCyciZFISH:ENSG00000132463-MONOMER.
ReactomeiR-HSA-192823. Viral mRNA Translation.

Names & Taxonomyi

Protein namesi
Recommended name:
G-rich sequence factor 1
Short name:
GRSF-1
Gene namesi
Name:GRSF1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

HGNCiHGNC:4610. GRSF1.

Subcellular locationi

  • Cytoplasm
  • Mitochondrion matrixmitochondrion nucleoid 1 Publication

  • Note: Localizes to mitochondrial RNA granules found in close proximity to the mitochondrial nucleoids.1 Publication

GO - Cellular componenti

  • cytoplasm Source: ProtInc
  • mitochondrial nucleoid Source: UniProtKB
  • mitochondrion Source: HPA
  • ribonucleoprotein granule Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Mitochondrion, Mitochondrion nucleoid

Pathology & Biotechi

Organism-specific databases

DisGeNETi2926.
OpenTargetsiENSG00000132463.
PharmGKBiPA29003.

Polymorphism and mutation databases

BioMutaiGRSF1.
DMDMi215274142.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 117MitochondrionSequence analysisAdd BLAST117
ChainiPRO_0000081597118 – 480G-rich sequence factor 1Add BLAST363

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei244PhosphoserineCombined sources1
Modified residuei335PhosphoserineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ12849.
MaxQBiQ12849.
PaxDbiQ12849.
PeptideAtlasiQ12849.
PRIDEiQ12849.

PTM databases

iPTMnetiQ12849.
PhosphoSitePlusiQ12849.

Expressioni

Gene expression databases

BgeeiENSG00000132463.
CleanExiHS_GRSF1.
ExpressionAtlasiQ12849. baseline and differential.
GenevisibleiQ12849. HS.

Organism-specific databases

HPAiHPA036984.
HPA036985.

Interactioni

Subunit structurei

Monomer. Found in a complex with DDX28, DHX30, FASTKD2 and FASTKD5 (PubMed:25683715). Interacts with the mitochondrial RNase P complex subunit TRMT10C/MRPP1.2 Publications

Protein-protein interaction databases

BioGridi109182. 53 interactors.
IntActiQ12849. 3 interactors.
STRINGi9606.ENSP00000254799.

Structurei

Secondary structure

1480
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi150 – 156Combined sources7
Helixi163 – 169Combined sources7
Turni170 – 172Combined sources3
Helixi178 – 181Combined sources4
Beta strandi182 – 186Combined sources5
Beta strandi192 – 202Combined sources11
Helixi203 – 210Combined sources8
Turni211 – 214Combined sources4
Beta strandi215 – 217Combined sources3
Beta strandi220 – 226Combined sources7
Helixi230 – 242Combined sources13
Beta strandi402 – 406Combined sources5
Helixi414 – 421Combined sources8
Beta strandi427 – 434Combined sources8
Beta strandi439 – 446Combined sources8
Helixi450 – 456Combined sources7
Helixi457 – 459Combined sources3
Beta strandi470 – 476Combined sources7

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2LMINMR-A140-245[»]
4QU6X-ray1.75A140-245[»]
4QU7X-ray2.50A/B/C/D400-480[»]
ProteinModelPortaliQ12849.
SMRiQ12849.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini122 – 246RRM 1PROSITE-ProRule annotationAdd BLAST125
Domaini250 – 326RRM 2PROSITE-ProRule annotationAdd BLAST77
Domaini401 – 480RRM 3PROSITE-ProRule annotationAdd BLAST80

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi55 – 111Ala-richAdd BLAST57

Sequence similaritiesi

Contains 3 RRM (RNA recognition motif) domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transit peptide

Phylogenomic databases

eggNOGiENOG410IQ3R. Eukaryota.
ENOG410YEKV. LUCA.
GeneTreeiENSGT00760000119102.
HOGENOMiHOG000220896.
HOVERGENiHBG055557.
InParanoidiQ12849.
OrthoDBiEOG091G0CTJ.
PhylomeDBiQ12849.
TreeFamiTF316157.

Family and domain databases

Gene3Di3.30.70.330. 3 hits.
InterProiIPR033106. GRSF1.
IPR012677. Nucleotide-bd_a/b_plait.
IPR000504. RRM_dom.
[Graphical view]
PANTHERiPTHR13976:SF42. PTHR13976:SF42. 1 hit.
PfamiPF00076. RRM_1. 1 hit.
[Graphical view]
SMARTiSM00360. RRM. 3 hits.
[Graphical view]
SUPFAMiSSF54928. SSF54928. 3 hits.
PROSITEiPS50102. RRM. 3 hits.
[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: Q12849-1) [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
MAGTRWVLGA LLRGCGCNCS SCRRTGAACL PFYSAAGSIP SGVSGRRRLL
60 70 80 90 100
LLLGAAAAAA SQTRGLQTGP VPPGRLAGPP AVATSAAAAA AASYSALRAS
110 120 130 140 150
LLPQSLAAAA AVPTRSYSQE SKTTYLEDLP PPPEYELAPS KLEEEVDDVF
160 170 180 190 200
LIRAQGLPWS CTMEDVLNFF SDCRIRNGEN GIHFLLNRDG KRRGDALIEM
210 220 230 240 250
ESEQDVQKAL EKHRMYMGQR YVEVYEINNE DVDALMKSLQ VKSSPVVNDG
260 270 280 290 300
VVRLRGLPYS CNEKDIVDFF AGLNIVDITF VMDYRGRRKT GEAYVQFEEP
310 320 330 340 350
EMANQALLKH REEIGNRYIE IFPSRRNEVR THVGSYKGKK IASFPTAKYI
360 370 380 390 400
TEPEMVFEEH EVNEDIQPMT AFESEKEIEL PKEVPEKLPE AADFGTTSSL
410 420 430 440 450
HFVHMRGLPF QANAQDIINF FAPLKPVRIT MEYSSSGKAT GEADVHFETH
460 470 480
EDAVAAMLKD RSHVHHRYIE LFLNSCPKGK
Length:480
Mass (Da):53,126
Last modified:November 25, 2008 - v3
Checksum:iE09EE4E70212A70F
GO
Isoform 2 (identifier: Q12849-5) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-162: Missing.

Note: No experimental confirmation available.
Show »
Length:318
Mass (Da):36,613
Checksum:iF0AB13A4797FFEBD
GO

Sequence cautioni

The sequence AAC95399 differs from that shown. Unlikely isoform. Aberrant splice sites.Curated
The sequence AAH40485 differs from that shown. Intron retention.Curated
The sequence AAY40942 differs from that shown. Reason: Erroneous gene model prediction.Curated
The sequence BAC03513 differs from that shown. Unlikely isoform. Aberrant splice sites.Curated
The sequence BAC86863 differs from that shown. Unlikely isoform. Aberrant splice sites.Curated
The sequence EAX05634 differs from that shown. Reason: Erroneous gene model prediction.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti264K → E in BAC86863 (PubMed:14702039).Curated1
Sequence conflicti365 – 366DI → VF in AAC95399 (PubMed:8036161).Curated2

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_047537277D → Y.1 PublicationCorresponds to variant rs17854012dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0431181 – 162Missing in isoform 2. 1 PublicationAdd BLAST162

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U07231 mRNA. Translation: AAC95399.1. Sequence problems.
AK056891 mRNA. Translation: BAG51822.1.
AK090755 mRNA. Translation: BAC03513.1. Sequence problems.
AK094806 mRNA. Translation: BAG52934.1.
AK095799 mRNA. Translation: BAG53131.1.
AK097055 mRNA. Translation: BAG53414.1.
AK127161 mRNA. Translation: BAC86863.1. Sequence problems.
AC021989 Genomic DNA. Translation: AAY40942.1. Sequence problems.
CH471057 Genomic DNA. Translation: EAX05631.1.
CH471057 Genomic DNA. Translation: EAX05634.1. Sequence problems.
BC040485 mRNA. Translation: AAH40485.1. Sequence problems.
CCDSiCCDS47069.1. [Q12849-1]
CCDS47070.1. [Q12849-5]
PIRiS48081.
RefSeqiNP_001091947.1. NM_001098477.1. [Q12849-5]
NP_002083.3. NM_002092.3.
XP_011530199.1. XM_011531897.2. [Q12849-5]
UniGeneiHs.309763.

Genome annotation databases

EnsembliENST00000254799; ENSP00000254799; ENSG00000132463. [Q12849-1]
ENST00000502323; ENSP00000425430; ENSG00000132463. [Q12849-5]
GeneIDi2926.
KEGGihsa:2926.
UCSCiuc010iia.2. human. [Q12849-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U07231 mRNA. Translation: AAC95399.1. Sequence problems.
AK056891 mRNA. Translation: BAG51822.1.
AK090755 mRNA. Translation: BAC03513.1. Sequence problems.
AK094806 mRNA. Translation: BAG52934.1.
AK095799 mRNA. Translation: BAG53131.1.
AK097055 mRNA. Translation: BAG53414.1.
AK127161 mRNA. Translation: BAC86863.1. Sequence problems.
AC021989 Genomic DNA. Translation: AAY40942.1. Sequence problems.
CH471057 Genomic DNA. Translation: EAX05631.1.
CH471057 Genomic DNA. Translation: EAX05634.1. Sequence problems.
BC040485 mRNA. Translation: AAH40485.1. Sequence problems.
CCDSiCCDS47069.1. [Q12849-1]
CCDS47070.1. [Q12849-5]
PIRiS48081.
RefSeqiNP_001091947.1. NM_001098477.1. [Q12849-5]
NP_002083.3. NM_002092.3.
XP_011530199.1. XM_011531897.2. [Q12849-5]
UniGeneiHs.309763.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2LMINMR-A140-245[»]
4QU6X-ray1.75A140-245[»]
4QU7X-ray2.50A/B/C/D400-480[»]
ProteinModelPortaliQ12849.
SMRiQ12849.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi109182. 53 interactors.
IntActiQ12849. 3 interactors.
STRINGi9606.ENSP00000254799.

PTM databases

iPTMnetiQ12849.
PhosphoSitePlusiQ12849.

Polymorphism and mutation databases

BioMutaiGRSF1.
DMDMi215274142.

Proteomic databases

EPDiQ12849.
MaxQBiQ12849.
PaxDbiQ12849.
PeptideAtlasiQ12849.
PRIDEiQ12849.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000254799; ENSP00000254799; ENSG00000132463. [Q12849-1]
ENST00000502323; ENSP00000425430; ENSG00000132463. [Q12849-5]
GeneIDi2926.
KEGGihsa:2926.
UCSCiuc010iia.2. human. [Q12849-1]

Organism-specific databases

CTDi2926.
DisGeNETi2926.
GeneCardsiGRSF1.
HGNCiHGNC:4610. GRSF1.
HPAiHPA036984.
HPA036985.
MIMi604851. gene.
neXtProtiNX_Q12849.
OpenTargetsiENSG00000132463.
PharmGKBiPA29003.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IQ3R. Eukaryota.
ENOG410YEKV. LUCA.
GeneTreeiENSGT00760000119102.
HOGENOMiHOG000220896.
HOVERGENiHBG055557.
InParanoidiQ12849.
OrthoDBiEOG091G0CTJ.
PhylomeDBiQ12849.
TreeFamiTF316157.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000132463-MONOMER.
ReactomeiR-HSA-192823. Viral mRNA Translation.

Miscellaneous databases

ChiTaRSiGRSF1. human.
GenomeRNAii2926.
PROiQ12849.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000132463.
CleanExiHS_GRSF1.
ExpressionAtlasiQ12849. baseline and differential.
GenevisibleiQ12849. HS.

Family and domain databases

Gene3Di3.30.70.330. 3 hits.
InterProiIPR033106. GRSF1.
IPR012677. Nucleotide-bd_a/b_plait.
IPR000504. RRM_dom.
[Graphical view]
PANTHERiPTHR13976:SF42. PTHR13976:SF42. 1 hit.
PfamiPF00076. RRM_1. 1 hit.
[Graphical view]
SMARTiSM00360. RRM. 3 hits.
[Graphical view]
SUPFAMiSSF54928. SSF54928. 3 hits.
PROSITEiPS50102. RRM. 3 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiGRSF1_HUMAN
AccessioniPrimary (citable) accession number: Q12849
Secondary accession number(s): B3KPW0
, Q4W5S5, Q6ZST3, Q8IWD6, Q8NBD2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: November 25, 2008
Last modified: November 2, 2016
This is version 147 of the entry and version 3 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

Miscellaneous

Depletion of GRSF1 by siRNA results in a combined OXPHOS assembly defect, with the prominent loss of complexes I, III, IV, and V. It also leads to altered steady-state levels of mitochondrial rRNAs and mRNAs.

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 4
    Human chromosome 4: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. 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.