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Q86SX6 (GLRX5_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 100. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (6) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Glutaredoxin-related protein 5, mitochondrial
Alternative name(s):
Monothiol glutaredoxin-5
Gene names
Name:GLRX5
Synonyms:C14orf87
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length157 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

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.

Subunit structure

Homodimer Probable. Ref.9

Subcellular location

Mitochondrion By similarity.

Involvement in disease

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. Ref.7

Sequence similarities

Belongs to the glutaredoxin family. Monothiol subfamily.

Contains 1 glutaredoxin domain.

Sequence caution

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

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 3131Mitochondrion Potential
Chain32 – 157126Glutaredoxin-related protein 5, mitochondrial
PRO_0000141650

Regions

Domain42 – 145104Glutaredoxin
Region97 – 1015Glutathione binding
Region122 – 1232Glutathione binding
Compositional bias8 – 114Poly-Ala
Compositional bias16 – 238Poly-Gly
Compositional bias33 – 408Poly-Gly

Sites

Metal binding671Iron-sulfur (2Fe-2S); shared with dimeric partner
Binding site591Glutathione
Binding site1091Glutathione; via amide nitrogen and carbonyl oxygen

Amino acid modifications

Modified residue591N6-succinyllysine By similarity

Natural variations

Natural variant1461A → T. Ref.1 Ref.6
Corresponds to variant rs11628901 [ dbSNP | Ensembl ].
VAR_026125

Secondary structure

..................... 157
Helix Strand Turn

Details...

Sequences

Sequence LengthMass (Da)Tools
Q86SX6 [UniParc].

Last modified February 1, 2005. Version 2.
Checksum: 5E6873BD5DE91F86

FASTA15716,628
        10         20         30         40         50         60 
MSGSLGRAAA ALLRWGRGAG GGGLWGPGVR AAGSGAGGGG SAEQLDALVK KDKVVVFLKG 

        70         80         90        100        110        120 
TPEQPQCGFS NAVVQILRLH GVRDYAAYNV LDDPELRQGI KDYSNWPTIP QVYLNGEFVG 

       130        140        150 
GCDILLQMHQ NGDLVEELKK LGIHSALLDE KKDQDSK 

« Hide

References

« Hide 'large scale' references
[1]"Deficiency of glutaredoxin 5 reveals Fe-S clusters are required for vertebrate haem synthesis."
The Tuebingen 2000 screen consortium
Wingert R.A., Galloway J.L., Barut B., Foott H., Fraenkel P., Axe J.L., Weber G.J., Dooley K., Davidson A.J., Schmid B., Paw B.H., Shaw G.C., Kingsley P., Palis J., Schubert H., Chen O., Kaplan J., Zon L.I.
Nature 436:1035-1039(2005) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT THR-146.
[2]Erratum
The Tuebingen 2000 screen consortium
Wingert R.A., Galloway J.L., Barut B., Foott H., Fraenkel P., Axe J.L., Weber G.J., Dooley K., Davidson A.J., Schmid B., Paw B.H., Shaw G.C., Kingsley P., Palis J., Schubert H., Chen O., Kaplan J.
Nature 437:920-920(2005)
[3]"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].
[4]"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.
[5]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]"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.
[7]"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.
[8]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
[9]"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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
CCDSCCDS9936.1.
RefSeqNP_057501.2. NM_016417.2.
UniGeneHs.744943.

3D structure databases

PDBe
RCSB-PDB
PDBj
EntryMethodResolution (Å)ChainPositionsPDBsum
2MMZNMR-A35-150[»]
2WULX-ray2.40A/B/C/D35-150[»]
ProteinModelPortalQ86SX6.
SMRQ86SX6. Positions 41-149.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid119386. 2 interactions.
IntActQ86SX6. 2 interactions.
STRING9606.ENSP00000328570.

PTM databases

PhosphoSiteQ86SX6.

Polymorphism databases

DMDM83288163.

Proteomic databases

MaxQBQ86SX6.
PaxDbQ86SX6.
PeptideAtlasQ86SX6.
PRIDEQ86SX6.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000331334; ENSP00000328570; ENSG00000182512.
GeneID51218.
KEGGhsa:51218.
UCSCuc001yem.1. human.

Organism-specific databases

CTD51218.
GeneCardsGC14P096001.
HGNCHGNC:20134. GLRX5.
HPAHPA042465.
MIM205950. phenotype.
609588. gene.
neXtProtNX_Q86SX6.
Orphanet255132. Adult-onset autosomal recessive sideroblastic anemia.
PharmGKBPA134992547.
GenAtlasSearch...

Phylogenomic databases

eggNOGCOG0278.
HOGENOMHOG000095211.
HOVERGENHBG051012.
InParanoidQ86SX6.
KOK07390.
OMATHMCISS.
OrthoDBEOG7B5WX3.
PhylomeDBQ86SX6.
TreeFamTF318988.

Gene expression databases

BgeeQ86SX6.
CleanExHS_GLRX5.
GenevestigatorQ86SX6.

Family and domain databases

Gene3D3.40.30.10. 1 hit.
InterProIPR002109. Glutaredoxin.
IPR004480. Monothiol_GRX-rel.
IPR012336. Thioredoxin-like_fold.
[Graphical view]
PANTHERPTHR10293. PTHR10293. 1 hit.
PfamPF00462. Glutaredoxin. 1 hit.
[Graphical view]
SUPFAMSSF52833. SSF52833. 1 hit.
TIGRFAMsTIGR00365. TIGR00365. 1 hit.
PROSITEPS51354. GLUTAREDOXIN_2. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

EvolutionaryTraceQ86SX6.
GeneWikiGLRX5.
GenomeRNAi51218.
NextBio54294.
PROQ86SX6.
SOURCESearch...

Entry information

Entry nameGLRX5_HUMAN
AccessionPrimary (citable) accession number: Q86SX6
Secondary accession number(s): Q0X088 expand/collapse secondary AC list , Q3YML0, Q86WY3, Q8IZ54
Entry history
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 statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

PDB cross-references

Index of Protein Data Bank (PDB) cross-references

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 14

Human chromosome 14: entries, gene names and cross-references to MIM