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Q86SX6

- GLRX5_HUMAN

UniProt

Q86SX6 - GLRX5_HUMAN

Protein

Glutaredoxin-related protein 5, mitochondrial

Gene

GLRX5

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 101 (01 Oct 2014)
      Sequence version 2 (01 Feb 2005)
      Previous versions | rss
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    Functioni

    Monothiol glutaredoxin involved in the biogenesis of iron-sulfur clusters. Required for normal iron homeostasis. Required for normal regulation of hemoglobin synthesis by the iron-sulfur protein ACO1.

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Binding sitei59 – 591Glutathione1 Publication
    Metal bindingi67 – 671Iron-sulfur (2Fe-2S); shared with dimeric partner
    Binding sitei109 – 1091Glutathione; via amide nitrogen and carbonyl oxygen1 Publication

    GO - Molecular functioni

    1. 2 iron, 2 sulfur cluster binding Source: UniProtKB-KW
    2. electron carrier activity Source: InterPro
    3. metal ion binding Source: UniProtKB-KW
    4. protein disulfide oxidoreductase activity Source: InterPro

    GO - Biological processi

    1. cell redox homeostasis Source: InterPro
    2. hemopoiesis Source: UniProtKB

    Keywords - Ligandi

    2Fe-2S, Iron, Iron-sulfur, Metal-binding

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Glutaredoxin-related protein 5, mitochondrial
    Alternative name(s):
    Monothiol glutaredoxin-5
    Gene namesi
    Name:GLRX5
    Synonyms:C14orf87
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 14

    Organism-specific databases

    HGNCiHGNC:20134. GLRX5.

    Subcellular locationi

    Mitochondrion By similarity

    GO - Cellular componenti

    1. mitochondrion Source: UniProtKB
    2. nucleus Source: HPA

    Keywords - Cellular componenti

    Mitochondrion

    Pathology & Biotechi

    Involvement in diseasei

    Anemia, sideroblastic, pyridoxine-refractory, autosomal recessive (PRARSA) [MIM:205950]: A form of sideroblastic anemia not responsive to pyridoxine. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus.1 Publication
    Note: The disease is caused by mutations affecting the gene represented in this entry.

    Organism-specific databases

    MIMi205950. phenotype.
    Orphaneti255132. Adult-onset autosomal recessive sideroblastic anemia.
    PharmGKBiPA134992547.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 3131MitochondrionSequence AnalysisAdd
    BLAST
    Chaini32 – 157126Glutaredoxin-related protein 5, mitochondrialPRO_0000141650Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei59 – 591N6-succinyllysineBy similarity

    Proteomic databases

    MaxQBiQ86SX6.
    PaxDbiQ86SX6.
    PeptideAtlasiQ86SX6.
    PRIDEiQ86SX6.

    PTM databases

    PhosphoSiteiQ86SX6.

    Expressioni

    Gene expression databases

    BgeeiQ86SX6.
    CleanExiHS_GLRX5.
    GenevestigatoriQ86SX6.

    Organism-specific databases

    HPAiHPA042465.

    Interactioni

    Subunit structurei

    Homodimer.1 Publication

    Protein-protein interaction databases

    BioGridi119386. 2 interactions.
    IntActiQ86SX6. 2 interactions.
    STRINGi9606.ENSP00000328570.

    Structurei

    Secondary structure

    1
    157
    Legend: HelixTurnBeta strand
    Show more details
    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Helixi42 – 5110
    Beta strandi52 – 609
    Beta strandi62 – 676
    Helixi68 – 7912
    Beta strandi86 – 894
    Helixi94 – 10411
    Beta strandi111 – 1144
    Beta strandi117 – 1204
    Helixi122 – 13110
    Helixi133 – 1408
    Turni146 – 1483

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    EntryMethodResolution (Å)ChainPositionsPDBsum
    2MMZNMR-A35-150[»]
    2WULX-ray2.40A/B/C/D35-150[»]
    ProteinModelPortaliQ86SX6.
    SMRiQ86SX6. Positions 41-149.
    ModBaseiSearch...
    MobiDBiSearch...

    Miscellaneous databases

    EvolutionaryTraceiQ86SX6.

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini42 – 145104GlutaredoxinPROSITE-ProRule annotationAdd
    BLAST

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni97 – 1015Glutathione binding
    Regioni122 – 1232Glutathione binding

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi8 – 114Poly-Ala
    Compositional biasi16 – 238Poly-Gly
    Compositional biasi33 – 408Poly-Gly

    Sequence similaritiesi

    Contains 1 glutaredoxin domain.PROSITE-ProRule annotation

    Keywords - Domaini

    Redox-active center, Transit peptide

    Phylogenomic databases

    eggNOGiCOG0278.
    HOGENOMiHOG000095211.
    HOVERGENiHBG051012.
    InParanoidiQ86SX6.
    KOiK07390.
    OMAiTHMCISS.
    OrthoDBiEOG7B5WX3.
    PhylomeDBiQ86SX6.
    TreeFamiTF318988.

    Family and domain databases

    Gene3Di3.40.30.10. 1 hit.
    InterProiIPR002109. Glutaredoxin.
    IPR004480. Monothiol_GRX-rel.
    IPR012336. Thioredoxin-like_fold.
    [Graphical view]
    PANTHERiPTHR10293. PTHR10293. 1 hit.
    PfamiPF00462. Glutaredoxin. 1 hit.
    [Graphical view]
    SUPFAMiSSF52833. SSF52833. 1 hit.
    TIGRFAMsiTIGR00365. TIGR00365. 1 hit.
    PROSITEiPS51354. GLUTAREDOXIN_2. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    Q86SX6-1 [UniParc]FASTAAdd to Basket

    « Hide

    MSGSLGRAAA ALLRWGRGAG GGGLWGPGVR AAGSGAGGGG SAEQLDALVK    50
    KDKVVVFLKG TPEQPQCGFS NAVVQILRLH GVRDYAAYNV LDDPELRQGI 100
    KDYSNWPTIP QVYLNGEFVG GCDILLQMHQ NGDLVEELKK LGIHSALLDE 150
    KKDQDSK 157
    Length:157
    Mass (Da):16,628
    Last modified:February 1, 2005 - v2
    Checksum:i5E6873BD5DE91F86
    GO

    Sequence cautioni

    The sequence CAD62364.1 differs from that shown. Reason: Erroneous initiation.

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti146 – 1461A → T.2 Publications
    Corresponds to variant rs11628901 [ dbSNP | Ensembl ].
    VAR_026125

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    DQ083331 mRNA. Translation: AAZ30731.1.
    BX248075 mRNA. Translation: CAD62364.1. Different initiation.
    AB223038 mRNA. Translation: BAF02301.1.
    CH471061 Genomic DNA. Translation: EAW81607.1.
    BC023528 mRNA. Translation: AAH23528.2.
    BC047680 mRNA. Translation: AAH47680.1.
    CCDSiCCDS9936.1.
    RefSeqiNP_057501.2. NM_016417.2.
    UniGeneiHs.744943.

    Genome annotation databases

    EnsembliENST00000331334; ENSP00000328570; ENSG00000182512.
    GeneIDi51218.
    KEGGihsa:51218.
    UCSCiuc001yem.1. human.

    Polymorphism databases

    DMDMi83288163.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    DQ083331 mRNA. Translation: AAZ30731.1 .
    BX248075 mRNA. Translation: CAD62364.1 . Different initiation.
    AB223038 mRNA. Translation: BAF02301.1 .
    CH471061 Genomic DNA. Translation: EAW81607.1 .
    BC023528 mRNA. Translation: AAH23528.2 .
    BC047680 mRNA. Translation: AAH47680.1 .
    CCDSi CCDS9936.1.
    RefSeqi NP_057501.2. NM_016417.2.
    UniGenei Hs.744943.

    3D structure databases

    Select the link destinations:
    PDBe
    RCSB PDB
    PDBj
    Links Updated
    Entry Method Resolution (Å) Chain Positions PDBsum
    2MMZ NMR - A 35-150 [» ]
    2WUL X-ray 2.40 A/B/C/D 35-150 [» ]
    ProteinModelPortali Q86SX6.
    SMRi Q86SX6. Positions 41-149.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 119386. 2 interactions.
    IntActi Q86SX6. 2 interactions.
    STRINGi 9606.ENSP00000328570.

    PTM databases

    PhosphoSitei Q86SX6.

    Polymorphism databases

    DMDMi 83288163.

    Proteomic databases

    MaxQBi Q86SX6.
    PaxDbi Q86SX6.
    PeptideAtlasi Q86SX6.
    PRIDEi Q86SX6.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000331334 ; ENSP00000328570 ; ENSG00000182512 .
    GeneIDi 51218.
    KEGGi hsa:51218.
    UCSCi uc001yem.1. human.

    Organism-specific databases

    CTDi 51218.
    GeneCardsi GC14P096001.
    HGNCi HGNC:20134. GLRX5.
    HPAi HPA042465.
    MIMi 205950. phenotype.
    609588. gene.
    neXtProti NX_Q86SX6.
    Orphaneti 255132. Adult-onset autosomal recessive sideroblastic anemia.
    PharmGKBi PA134992547.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG0278.
    HOGENOMi HOG000095211.
    HOVERGENi HBG051012.
    InParanoidi Q86SX6.
    KOi K07390.
    OMAi THMCISS.
    OrthoDBi EOG7B5WX3.
    PhylomeDBi Q86SX6.
    TreeFami TF318988.

    Miscellaneous databases

    EvolutionaryTracei Q86SX6.
    GeneWikii GLRX5.
    GenomeRNAii 51218.
    NextBioi 54294.
    PROi Q86SX6.
    SOURCEi Search...

    Gene expression databases

    Bgeei Q86SX6.
    CleanExi HS_GLRX5.
    Genevestigatori Q86SX6.

    Family and domain databases

    Gene3Di 3.40.30.10. 1 hit.
    InterProi IPR002109. Glutaredoxin.
    IPR004480. Monothiol_GRX-rel.
    IPR012336. Thioredoxin-like_fold.
    [Graphical view ]
    PANTHERi PTHR10293. PTHR10293. 1 hit.
    Pfami PF00462. Glutaredoxin. 1 hit.
    [Graphical view ]
    SUPFAMi SSF52833. SSF52833. 1 hit.
    TIGRFAMsi TIGR00365. TIGR00365. 1 hit.
    PROSITEi PS51354. GLUTAREDOXIN_2. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT THR-146.
    2. "Biological function of human glutaredoxin 3 (Grx 3), a novel mitochondrial monothiol Grx."
      Kurosawa N., Isobe M., Saito M.
      Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    3. "Full-length cDNA libraries and normalization."
      Li W.B., Gruber C., Jessee J., Polayes D.
      Submitted (FEB-2003) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: B-cell.
    4. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    5. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT THR-146.
      Tissue: Skin and Testis.
    6. "The human counterpart of zebrafish shiraz shows sideroblastic-like microcytic anemia and iron overload."
      Camaschella C., Campanella A., De Falco L., Boschetto L., Merlini R., Silvestri L., Levi S., Iolascon A.
      Blood 110:1353-1358(2007) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN PRARSA.
    7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    8. "The crystal structure of human GLRX5: iron-sulfur cluster co-ordination, tetrameric assembly and monomer activity."
      Johansson C., Roos A.K., Montano S.J., Sengupta R., Filippakopoulos P., Guo K., von Delft F., Holmgren A., Oppermann U., Kavanagh K.L.
      Biochem. J. 433:303-311(2011) [PubMed] [Europe PMC] [Abstract]
      Cited for: X-RAY CRYSTALLOGRAPHY (2.4 ANGSTROMS) OF 35-150 IN COMPLEX WITH GLUTATHIONE AND IRON-SULFUR CLUSTER, SUBUNIT.

    Entry informationi

    Entry nameiGLRX5_HUMAN
    AccessioniPrimary (citable) accession number: Q86SX6
    Secondary accession number(s): Q0X088
    , Q3YML0, Q86WY3, Q8IZ54
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: February 1, 2005
    Last sequence update: February 1, 2005
    Last modified: October 1, 2014
    This is version 101 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

    3D-structure, Complete proteome, Reference proteome

    Documents

    1. Human chromosome 14
      Human chromosome 14: 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

    External Data

    Dasty 3