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Q86SX6

- GLRX5_HUMAN

UniProt

Q86SX6 - GLRX5_HUMAN

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Protein

Glutaredoxin-related protein 5, mitochondrial

Gene
GLRX5, C14orf87
Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

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 – 591Glutathione
Metal bindingi67 – 671Iron-sulfur (2Fe-2S); shared with dimeric partner
Binding sitei109 – 1091Glutathione; via amide nitrogen and carbonyl oxygen

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
Complete GO annotation...

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
Complete GO annotation...

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.
Note: The disease is caused by mutations affecting the gene represented in this entry.1 Publication

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 – 3131Mitochondrion Reviewed predictionAdd
BLAST
Chaini32 – 157126Glutaredoxin-related protein 5, mitochondrialPRO_0000141650Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei59 – 591N6-succinyllysine By 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 Inferred.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.

Miscellaneous databases

EvolutionaryTraceiQ86SX6.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini42 – 145104GlutaredoxinAdd
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

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

« Hide 'large scale' publications
  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: July 9, 2014
This is version 100 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

Similar proteinsi