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    DGCR2 DiGeorge syndrome critical region gene 2 [ Homo sapiens ]

    Gene ID: 9993, updated on 11-May-2012

    Summary

    Official Symbol
    DGCR2provided by HGNC
    Official Full Name
    DiGeorge syndrome critical region gene 2provided by HGNC
    Primary source
    HGNC:2845
    See related
    Ensembl:ENSG00000070413; HPRD:08999; MIM:600594; Vega:OTTHUMG00000150141
    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
    IDD; LAN; DGS-C; SEZ-12; KIAA0163; DKFZp686I1730
    Summary
    Deletions of the 22q11.2 have been associated with a wide range of developmental defects (notably DiGeorge syndrome, velocardiofacial syndrome, conotruncal anomaly face syndrome and isolated conotruncal cardiac defects) classified under the acronym CATCH 22. The DGCR2 gene encodes a novel putative adhesion receptor protein, which could play a role in neural crest cells migration, a process which has been proposed to be altered in DiGeorge syndrome. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010]

    Genomic context

    Location :
    22q11.21
    Sequence :
    Chromosome: 22; NC_000022.10 (19023795..19109967, complement)
    See DGCR2 in Epigenomics, MapViewer

    Chromosome 22 - NC_000022.10Genomic Context describing neighboring genes Neighboring gene uncharacterized LOC100506454 Neighboring gene DiGeorge syndrome critical region gene 10 (non-protein coding) Neighboring gene hCG1646661-like Neighboring gene ribosomal protein L28 pseudogene Neighboring gene DiGeorge syndrome critical region gene 11 (non-protein coding) Neighboring gene testis-specific serine kinase 1A pseudogene Neighboring gene DiGeorge syndrome critical region gene 14 Neighboring gene testis-specific serine kinase 2

    Genomic regions, transcripts, and products

    Bibliography

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

    Interactions

    Products Interactant Other Gene Complex Source Pubs Description
    P98153 Q9HAU4 SMURF2    HPRD  PubMed  

    General gene information

    Markers

    Homology

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    binding IEA
    Inferred from Electronic Annotation
    more info
     
    receptor activity IEA
    Inferred from Electronic Annotation
    more info
     
    sugar binding IEA
    Inferred from Electronic Annotation
    more info
     
    Process Evidence Code Pubs
    cell adhesion IEA
    Inferred from Electronic Annotation
    more info
     
    organ morphogenesis TAS
    Traceable Author Statement
    more info
    PubMed 
    Component Evidence Code Pubs
    integral to membrane IEA
    Inferred from Electronic Annotation
    more info
     
    membrane IEA
    Inferred from Electronic Annotation
    more info
     

    General protein information

    Preferred Names
    integral membrane protein DGCR2/IDD
    Names
    integral membrane protein DGCR2/IDD
    DiGeorge syndrome critical region protein 2
    integral membrane protein deleted in DiGeorge syndrome

    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_021333.1 RefSeqGene

      Range
      5001..91173
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001173533.1NP_001167004.1  integral membrane protein DGCR2/IDD isoform 2 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) lacks an alternate in-frame exon in the 5' coding region, compared to variant 1. The resulting isoform (2) lacks an internal segment, compared to isoform 1.
      Source sequence(s)
      AK304382, AW502246, BC040500, DA494775
      Consensus CDS
      CCDS54496.1
      UniProtKB/Swiss-Prot
      P98153
      UniProtKB/TrEMBL
      Q8IWC8
      Related
      ENSP00000440062, ENST00000537045
      Conserved Domains (2) summary
      cd03599
      Location:74226
      Blast Score: 849
      CLECT_DGCR2_like; C-type lectin-like domain (CTLD) of the type found in DGCR2, an integral membrane protein deleted in DiGeorge Syndrome (DGS)
      cl02515
      Location:230288
      Blast Score: 126
      VWC; von Willebrand factor type C domain
    2. NM_001173534.1NP_001167005.1  integral membrane protein DGCR2/IDD isoform 3 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (3) lacks an alternate in-frame exon and uses an alternate in-frame splice site in the 5' coding region, compared to variant 1. The resulting isoform (3) lacks two internal segments, compared to isoform 1.
      Source sequence(s)
      AK296337, AW502246, BC032430, BC040500, DA494775
      UniProtKB/TrEMBL
      B7Z3T5
      UniProtKB/Swiss-Prot
      P98153
      UniProtKB/TrEMBL
      Q8IWC8
      Conserved Domains (2) summary
      cd03599
      Location:71223
      Blast Score: 848
      CLECT_DGCR2_like; C-type lectin-like domain (CTLD) of the type found in DGCR2, an integral membrane protein deleted in DiGeorge Syndrome (DGS)
      cl02515
      Location:227285
      Blast Score: 126
      VWC; von Willebrand factor type C domain
    3. NM_001184781.1NP_001171710.1  integral membrane protein DGCR2/IDD isoform 4 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (4) uses an alternate in-frame splice site in the 5' coding region, compared to variant 1. The resulting isoform (4) lacks a short internal segment, compared to isoform 1.
      Source sequence(s)
      AK296337, AW502246, CR936871, DA494775
      UniProtKB/TrEMBL
      B7Z3T5
      UniProtKB/TrEMBL
      Q5CZ70
      Conserved Domains (3) summary
      cd00112
      Location:3066
      Blast Score: 122
      LDLa; Low Density Lipoprotein Receptor Class A domain, a cysteine-rich repeat that plays a central role in mammalian cholesterol metabolism; the receptor protein binds LDL and transports it into cells by endocytosis; 7 successive cysteine-rich repeats of about ...
      cd03599
      Location:112264
      Blast Score: 840
      CLECT_DGCR2_like; C-type lectin-like domain (CTLD) of the type found in DGCR2, an integral membrane protein deleted in DiGeorge Syndrome (DGS)
      cl02515
      Location:268326
      Blast Score: 119
      VWC; von Willebrand factor type C domain
    4. NM_005137.2NP_005128.1  integral membrane protein DGCR2/IDD isoform 1 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (1).
      Source sequence(s)
      AW502246, BC040500, D79985, DA494775
      Consensus CDS
      CCDS33598.1
      UniProtKB/Swiss-Prot
      P98153
      UniProtKB/TrEMBL
      Q8IWC8
      Related
      ENSP00000263196, OTTHUMP00000196406, ENST00000263196, OTTHUMT00000316504
      Conserved Domains (3) summary
      cd00112
      Location:3066
      Blast Score: 122
      LDLa; Low Density Lipoprotein Receptor Class A domain, a cysteine-rich repeat that plays a central role in mammalian cholesterol metabolism; the receptor protein binds LDL and transports it into cells by endocytosis; 7 successive cysteine-rich repeats of about ...
      cd03599
      Location:115267
      Blast Score: 841
      CLECT_DGCR2_like; C-type lectin-like domain (CTLD) of the type found in DGCR2, an integral membrane protein deleted in DiGeorge Syndrome (DGS)
      cl02515
      Location:271329
      Blast Score: 120
      VWC; von Willebrand factor type C domain

    RNA

    1. NR_033674.1 RNA Sequence

      Description
      Transcript Variant: This variant (5) lacks an alternate in-frame exon and uses an alternate in-frame splice site in the 5' coding region, compared to variant 1, which results in a frameshift and early stop codon. The transcript is sufficiently abundant to represent as a RefSeq record; however, the predicted protein is not represented because the product is significantly truncated and the transcript is a candidate for nonsense-mediated mRNA decay (NMD).
      Source sequence(s)
      AK295697, AW502246, CR936871, DA494775

    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_000022.10 Reference GRCh37.p5 Primary Assembly

      Range
      19023795..19109967, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate HuRef

    Genomic

    1. AC_000154.1 Alternate HuRef

      Range
      2645051..2731450, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Related Sequences

    Nucleotide Protein
    Heading Accession and Version
    genomic AC000095.5 (35000..43727) None
    genomic AC004461.3 (15684..44872) None
    genomic AC004462.4 (6721..36015) None
    genomic AC004471.2 (1008..19902) None
    genomic CH471176.2 EAX03061.1
      EAX03062.1
      EAX03063.1
    mRNA AK291670.1 BAF84359.1
    mRNA AK295697.1 BAH12160.1
    mRNA AK296337.1 BAH12321.1
    mRNA AK303130.1 BAH13903.1
    mRNA AK304382.1 BAH14171.1
    mRNA AW502246.1 None
    mRNA BC020339.1 None
    mRNA BC032430.1 AAH32430.1
    mRNA BC035425.1 None
    mRNA BC040500.1 AAH40500.1
    mRNA CR456433.1 CAG30319.1
    mRNA CR936871.1 CAI59783.1
    mRNA D79985.1 BAA11480.1
    mRNA DA494775.1 None
    mRNA FJ032369.1 ACI00235.1
    mRNA L46352.1 AAB59561.1
    mRNA L77560.1 None
    mRNA X83545.1 CAA58536.1
    mRNA X84076.1 CAA58883.1
    other-genetic CU012993.1 CAK54424.1
    other-genetic CU013281.1 CAK54723.1
    other-genetic DQ894160.2 ABM85086.1
    Protein Accession Links
    GenPept Link UniProtKB Link
    P98153.1 GenPept UniProtKB/Swiss-Prot:P98153
    Q5CZ70 GenPept UniProtKB/TrEMBL:Q5CZ70
    Q8IWC8 GenPept UniProtKB/TrEMBL:Q8IWC8

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