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    FMR1 fragile X mental retardation 1 [ Homo sapiens ]

    Gene ID: 2332, updated on 20-May-2012

    Summary

    Official Symbol
    FMR1provided by HGNC
    Official Full Name
    fragile X mental retardation 1provided by HGNC
    Primary source
    HGNC:3775
    See related
    Ensembl:ENSG00000102081; HPRD:02398; MIM:309550; Vega:OTTHUMG00000022606
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    POF; FMRP; POF1; FRAXA; MGC87458
    Summary
    The protein encoded by this gene binds RNA and is associated with polysomes. The encoded protein may be involved in mRNA trafficking from the nucleus to the cytoplasm. A trinucleotide repeat (CGG) in the 5' UTR is normally found at 6-53 copies, but an expansion to 55-230 repeats is the cause of fragile X syndrome. Expansion of the trinucleotide repeat may also cause one form of premature ovarian failure (POF1). Multiple alternatively spliced transcript variants that encode different protein isoforms and which are located in different cellular locations have been described for this gene. [provided by RefSeq, May 2010]

    Genomic context

    Location :
    Xq27.3
    Sequence :
    Chromosome: X; NC_000023.10 (146993469..147032647)
    See FMR1 in Epigenomics, MapViewer

    Chromosome X - NC_000023.10Genomic Context describing neighboring genes Neighboring gene microRNA 514a-3 Neighboring gene SRA stem-loop interacting RNA binding protein pseudogene Neighboring gene FMR1 antisense RNA 1 (non-protein coding) Neighboring gene fragile X mental retardation 1 neighbor Neighboring gene ferritin, heavy polypeptide 1 pseudogene 8

    Genomic regions, transcripts, and products

    Bibliography

    GeneRIFs: Gene References Into Functions What's a GeneRIF?

    Interactions

    Products Interactant Other Gene Complex Source Pubs Description
    NC_000023.8     BIND  PubMed The MeCP2 corepressor complex interacts with the hypermethylated FMR1 promoter. 
    NP_002015.1 NM_005909.2 MAP1B    BIND  PubMed FMR1 (FMRP) interacts with MAP1B mRNA. This interaction was modeled on a demonstrated interaction between FMR1 from an unspecified species and human MAP1B mRNA. 
    NP_002015.1 NP_005484.2 RANBP9    BIND  PubMed FMR1 (FMRP) interacts with RANBP9 (RanBPM). 
    Q06787 Q7L576 CYFIP1    HPRD  PubMed  
    Q06787 Q96F07 CYFIP2    HPRD  PubMed  
    Q06787 P51114 FXR1    HPRD  PubMed  
    Q06787 P51116 FXR2    HPRD  PubMed  
    Q06787 P19338 NCL    HPRD  PubMed  
    Q06787 Q9UHK0 NUFIP1    HPRD  PubMed  
    Q06787 Q7Z417 NUFIP2    HPRD  PubMed  
    Q06787 Q96S59 RANBP9    HPRD  PubMed  
    Q06787 O95793 STAU1    HPRD  PubMed  
    Q06787 P63279 UBE2I    HPRD  PubMed  
    Q06787 P67809 YBX1    HPRD  PubMed  
    BioGRID:108619 BioGRID:115535 CALCOCO2    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:108619 BioGRID:116800 CYFIP1    BioGRID  PubMed Affinity Capture-Western; Reconstituted Complex; Two-hybrid 
    BioGRID:108619 BioGRID:117945 CYFIP2    BioGRID  PubMed Reconstituted Complex; Two-hybrid 
    BioGRID:108619 BioGRID:108619 FMR1    BioGRID  PubMed Affinity Capture-Western; Two-hybrid 
    BioGRID:108619 BioGRID:113760 FXR1    BioGRID  PubMed Co-purification; Reconstituted Complex; Two-hybrid 
    BioGRID:108619 BioGRID:114890 FXR2    BioGRID  PubMed Affinity Capture-Western; Co-purification; Reconstituted Complex; Two-hybrid 
    BioGRID:108619 BioGRID:110771 NCL    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:108619 BioGRID:117807 NUFIP1    BioGRID  PubMed Affinity Capture-Western; Reconstituted Complex; Two-hybrid 
    BioGRID:108619 BioGRID:121591 NUFIP2    BioGRID  PubMed Affinity Capture-Western; Reconstituted Complex; Two-hybrid 
    BioGRID:108619 BioGRID:111549 PRKAA1    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:108619 BioGRID:112657 STAU1    BioGRID  PubMed Affinity Capture-Western 
    BioGRID:108619 BioGRID:118878 TBK1    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:108619 BioGRID:115103 TBKBP1    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:108619 BioGRID:113164 UBC    BioGRID  PubMed Affinity Capture-MS 
    BioGRID:108619 BioGRID:113177 UBE2I    BioGRID  PubMed Two-hybrid 
    BioGRID:108619 BioGRID:123349 ZBP1    BioGRID  PubMed Affinity Capture-MS 

    General gene information

    Markers

    Homology

    Pathways from BioSystems

    • RNA transport, organism-specific biosystem (from KEGG)
      RNA transport, organism-specific biosystemRNA transport from the nucleus to the cytoplasm is fundamental for gene expression. The different RNA species that are produced in the nucleus are exported through the nuclear pore complexes (NPCs) ...
    • RNA transport, conserved biosystem (from KEGG)
      RNA transport, conserved biosystemRNA transport from the nucleus to the cytoplasm is fundamental for gene expression. The different RNA species that are produced in the nucleus are exported through the nuclear pore complexes (NPCs) ...

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    RNA binding IEA
    Inferred from Electronic Annotation
    more info
     
    RNA binding TAS
    Traceable Author Statement
    more info
    PubMed 
    mRNA binding TAS
    Traceable Author Statement
    more info
    PubMed 
    protein binding IPI
    Inferred from Physical Interaction
    more info
    PubMed 
    Process Evidence Code Pubs
    central nervous system development IEA
    Inferred from Electronic Annotation
    more info
     
    mRNA transport IEA
    Inferred from Electronic Annotation
    more info
     
    negative regulation of translational initiation ISS
    Inferred from Sequence or Structural Similarity
    more info
     
    transport IEA
    Inferred from Electronic Annotation
    more info
     
    Component Evidence Code Pubs
    cytoplasm TAS
    Traceable Author Statement
    more info
    PubMed 
    mRNA cap binding complex ISS
    Inferred from Sequence or Structural Similarity
    more info
     
    nucleolus TAS
    Traceable Author Statement
    more info
    PubMed 
    nucleoplasm TAS
    Traceable Author Statement
    more info
    PubMed 
    nucleus IEA
    Inferred from Electronic Annotation
    more info
     
    colocalizes_with polysomal ribosome TAS
    Traceable Author Statement
    more info
    PubMed 
    soluble fraction TAS
    Traceable Author Statement
    more info
    PubMed 

    General protein information

    Preferred Names
    fragile X mental retardation protein 1
    Names
    fragile X mental retardation protein 1
    protein FMR-1
    fragile X mental retardation 1 protein

    NCBI Reference Sequences (RefSeq)

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    Genomic

    1. NG_007529.1 RefSeqGene

      Range
      4961..44139
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001185075.1NP_001172004.1  fragile X mental retardation protein 1 isoform ISO6

      Status: REVIEWED

      Description
      Transcript Variant: This variant (ISO6) lacks an alternate segment and uses a different splice site in the 3' coding region which shifts the reading frame, compared to variant ISO1. The resulting protein (isoform ISO6) has a shorter and distinct C-terminus when it is compared to isoform ISO1.
      Source sequence(s)
      BC086957, BQ213316, BX498094, CK825936, L29074
      Consensus CDS
      CCDS55518.1
      UniProtKB/TrEMBL
      G8JLE9
      UniProtKB/Swiss-Prot
      Q06787
      Related
      ENSP00000359502, ENST00000370471
      Conserved Domains (2) summary
      cl00098
      Location:283326
      Blast Score: 104
      KH-II; KH-II (K homology RNA-binding domain, type II). KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins (e.g. ribosomal protein S3), transcription factors (e.g. NusA_K), and post-transcriptional ...
      cl15261
      Location:59117
      Blast Score: 113
      CHROMO; Chromatin organization modifier (chromo) domain is a conserved region of around 50 amino acids found in a variety of chromosomal proteins, which appear to play a role in the functional organization of the eukaryotic nucleus. Experimental evidence ...
    2. NM_001185076.1NP_001172005.1  fragile X mental retardation protein 1 isoform ISO7

      Status: REVIEWED

      Description
      Transcript Variant: This variant (ISO7) lacks an alternate segment, compared to variant ISO1. The resulting protein (isoform ISO7) is shorter when it is compared to isoform ISO1.
      Source sequence(s)
      BC086957, BQ213316, BQ417290, CN315330, CX871385, L29074
      Consensus CDS
      CCDS55519.1
      UniProtKB/TrEMBL
      G8JL90
      UniProtKB/Swiss-Prot
      Q06787
      Related
      ENSP00000218200, ENST00000218200
      Conserved Domains (4) summary
      cd00105
      Location:286332
      Blast Score: 99
      KH-I; K homology RNA-binding domain, type I. KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins, transcription factors and post-transcriptional modifiers of mRNA. There are two different KH domains that ...
      pfam12235
      Location:406460
      Blast Score: 199
      FXR1P_C; Fragile X-related 1 protein C terminal
      cl00098
      Location:221276
      Blast Score: 96
      KH-II; KH-II (K homology RNA-binding domain, type II). KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins (e.g. ribosomal protein S3), transcription factors (e.g. NusA_K), and post-transcriptional ...
      cl15261
      Location:59117
      Blast Score: 114
      CHROMO; Chromatin organization modifier (chromo) domain is a conserved region of around 50 amino acids found in a variety of chromosomal proteins, which appear to play a role in the functional organization of the eukaryotic nucleus. Experimental evidence ...
    3. NM_001185081.1NP_001172010.1  fragile X mental retardation protein 1 isoform ISO12

      Status: REVIEWED

      Description
      Transcript Variant: This variant (ISO12) lacks two alternate segments and uses a different splice site which changes the reading frame, compared to variant ISO1. The resulting protein (isoform ISO12) has a shorter and distinct C-terminus when it is compared to isoform ISO1.
      Source sequence(s)
      BC086957, BQ213316, BQ287827, CK825936, L29074
      UniProtKB/Swiss-Prot
      Q06787
      Conserved Domains (2) summary
      cl00098
      Location:283326
      Blast Score: 103
      KH-II; KH-II (K homology RNA-binding domain, type II). KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins (e.g. ribosomal protein S3), transcription factors (e.g. NusA_K), and post-transcriptional ...
      cl15261
      Location:59117
      Blast Score: 114
      CHROMO; Chromatin organization modifier (chromo) domain is a conserved region of around 50 amino acids found in a variety of chromosomal proteins, which appear to play a role in the functional organization of the eukaryotic nucleus. Experimental evidence ...
    4. NM_001185082.1NP_001172011.1  fragile X mental retardation protein 1 isoform ISO9

      Status: REVIEWED

      Description
      Transcript Variant: This variant (ISO9) lacks an alternate segment and uses a different splice site in the 3' coding region, compared to variant ISO1. The resulting protein (isoform ISO9) is shorter when it is compared to isoform ISO1.
      Source sequence(s)
      BC086957, L29074
      UniProtKB/Swiss-Prot
      Q06787
      Conserved Domains (3) summary
      pfam12235
      Location:406531
      Blast Score: 212
      FXR1P_C; Fragile X-related 1 protein C terminal
      cl00098
      Location:283326
      Blast Score: 108
      KH-II; KH-II (K homology RNA-binding domain, type II). KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins (e.g. ribosomal protein S3), transcription factors (e.g. NusA_K), and post-transcriptional ...
      cl15261
      Location:59117
      Blast Score: 114
      CHROMO; Chromatin organization modifier (chromo) domain is a conserved region of around 50 amino acids found in a variety of chromosomal proteins, which appear to play a role in the functional organization of the eukaryotic nucleus. Experimental evidence ...
    5. NM_002024.5NP_002015.1  fragile X mental retardation protein 1 isoform ISO1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (ISO1) represents the longest transcript and it encodes the longest protein (isoform ISO1).
      Source sequence(s)
      BC086957, L29074, X69962
      Consensus CDS
      CCDS14682.1
      UniProtKB/Swiss-Prot
      Q06787
      Related
      ENSP00000359506, OTTHUMP00000024197, ENST00000370475, OTTHUMT00000058655
      Conserved Domains (4) summary
      cd00105
      Location:286332
      Blast Score: 99
      KH-I; K homology RNA-binding domain, type I. KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins, transcription factors and post-transcriptional modifiers of mRNA. There are two different KH domains that ...
      pfam12235
      Location:427481
      Blast Score: 200
      FXR1P_C; Fragile X-related 1 protein C terminal
      cl00098
      Location:221276
      Blast Score: 96
      KH-II; KH-II (K homology RNA-binding domain, type II). KH binds single-stranded RNA or DNA. It is found in a wide variety of proteins including ribosomal proteins (e.g. ribosomal protein S3), transcription factors (e.g. NusA_K), and post-transcriptional ...
      cl15261
      Location:59117
      Blast Score: 114
      CHROMO; Chromatin organization modifier (chromo) domain is a conserved region of around 50 amino acids found in a variety of chromosomal proteins, which appear to play a role in the functional organization of the eukaryotic nucleus. Experimental evidence ...

    RNA

    1. NR_033699.1 RNA Sequence

      Description
      Transcript Variant: This variant (ISO4) lacks an alternate segment, compared to variant ISO1. This variant is represented as non-coding because the use of the 5'-most translational start codon, as used in variant ISO1, renders this transcript a candidate for nonsense-mediated mRNA decay (NMD).
      Source sequence(s)
      BC086957, BQ213316, BX498094, CK825936, L29074
    2. NR_033700.1 RNA Sequence

      Description
      Transcript Variant: This variant (ISO10) lacks two alternate segments, compared to variant ISO1. This variant is represented as non-coding because the use of the 5'-most translational start codon, as used in variant ISO1, renders this transcript a candidate for nonsense-mediated mRNA decay (NMD).
      Source sequence(s)
      BC086957, BQ213316, CK825936, L29074

    RefSeqs of Annotated Genomes: Build 37.3

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCh37.p5 Primary Assembly

    Genomic

    1. NC_000023.10 Reference GRCh37.p5 Primary Assembly

      Range
      146993469..147032647
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate HuRef

    Genomic

    1. AC_000155.1 Alternate HuRef

      Range
      135963879..135993282
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

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