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Protein

Glyoxylate reductase/hydroxypyruvate reductase

Gene

GRHPR

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

Functioni

Enzyme with hydroxy-pyruvate reductase, glyoxylate reductase and D-glycerate dehydrogenase enzymatic activities. Reduces hydroxypyruvate to D-glycerate, glyoxylate to glycolate oxidizes D-glycerate to hydroxypyruvate.

Catalytic activityi

Glycolate + NADP+ = glyoxylate + NADPH.
D-glycerate + NAD(P)+ = hydroxypyruvate + NAD(P)H.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei217NADP1 Publication1
Binding sitei243NADP; via carbonyl oxygen1 Publication1
Binding sitei245Substrate1 Publication1
Binding sitei269Substrate1 Publication1
Sitei274Raises pKa of active site His1 Publication1
Active sitei293Proton donor1 Publication1
Binding sitei295NADP; via amide nitrogen1 Publication1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi162 – 164NADP1 Publication3
Nucleotide bindingi185 – 188NADP1 Publication4

GO - Molecular functioni

  • carboxylic acid binding Source: Ensembl
  • glycerate dehydrogenase activity Source: UniProtKB
  • glyoxylate reductase (NADP) activity Source: UniProtKB
  • hydroxypyruvate reductase activity Source: UniProtKB
  • NAD binding Source: UniProtKB
  • NADPH binding Source: UniProtKB
  • protein homodimerization activity Source: UniProtKB

GO - Biological processi

  • dicarboxylic acid metabolic process Source: Ensembl
  • excretion Source: UniProtKB
  • glyoxylate metabolic process Source: Reactome
  • metabolic process Source: UniProtKB
  • oxidation-reduction process Source: UniProtKB
  • protein oligomerization Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

NADP

Enzyme and pathway databases

BioCyciZFISH:HS06275-MONOMER.
ReactomeiR-HSA-389661. Glyoxylate metabolism and glycine degradation.
SABIO-RKQ9UBQ7.

Names & Taxonomyi

Protein namesi
Recommended name:
Glyoxylate reductase/hydroxypyruvate reductase (EC:1.1.1.79, EC:1.1.1.81)
Gene namesi
Name:GRHPR
Synonyms:GLXR
ORF Names:MSTP035
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 9

Organism-specific databases

HGNCiHGNC:4570. GRHPR.

Subcellular locationi

GO - Cellular componenti

  • cytoplasm Source: LIFEdb
  • cytosol Source: GO_Central
  • extracellular exosome Source: UniProtKB
  • peroxisomal matrix Source: Reactome
Complete GO annotation...

Pathology & Biotechi

Involvement in diseasei

Hyperoxaluria primary 2 (HP2)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA disorder characterized by elevated urinary excretion of oxalate and L-glycerate, progressive tissue accumulation of insoluble calcium oxalate, nephrolithiasis, nephrocalcinosis, and end-stage renal disease.
See also OMIM:260000

Organism-specific databases

DisGeNETi9380.
MalaCardsiGRHPR.
MIMi260000. phenotype.
OpenTargetsiENSG00000137106.
Orphaneti93599. Primary hyperoxaluria type 2.
PharmGKBiPA28965.

Polymorphism and mutation databases

BioMutaiGRHPR.
DMDMi47116943.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000759441 – 328Glyoxylate reductase/hydroxypyruvate reductaseAdd BLAST328

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei36PhosphoserineCombined sources1
Modified residuei272PhosphoserineCombined sources1
Modified residuei298PhosphothreonineCombined sources1

Keywords - PTMi

Phosphoprotein

Proteomic databases

EPDiQ9UBQ7.
PaxDbiQ9UBQ7.
PeptideAtlasiQ9UBQ7.
PRIDEiQ9UBQ7.

2D gel databases

REPRODUCTION-2DPAGEIPI00037448.
UCD-2DPAGEQ9UBQ7.

PTM databases

iPTMnetiQ9UBQ7.
PhosphoSitePlusiQ9UBQ7.
SwissPalmiQ9UBQ7.

Expressioni

Tissue specificityi

Ubiquitous. Most abundantly expressed in the liver.1 Publication

Gene expression databases

BgeeiENSG00000137106.
CleanExiHS_GRHPR.
ExpressionAtlasiQ9UBQ7. baseline and differential.
GenevisibleiQ9UBQ7. HS.

Organism-specific databases

HPAiHPA022971.

Interactioni

Subunit structurei

Homodimer.2 Publications

GO - Molecular functioni

  • protein homodimerization activity Source: UniProtKB

Protein-protein interaction databases

BioGridi114781. 32 interactors.
IntActiQ9UBQ7. 5 interactors.
MINTiMINT-5005010.
STRINGi9606.ENSP00000313432.

Structurei

Secondary structure

1328
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi7 – 13Combined sources7
Helixi17 – 25Combined sources9
Beta strandi29 – 33Combined sources5
Beta strandi36 – 38Combined sources3
Helixi42 – 49Combined sources8
Beta strandi53 – 57Combined sources5
Helixi65 – 71Combined sources7
Beta strandi77 – 83Combined sources7
Helixi90 – 95Combined sources6
Beta strandi99 – 101Combined sources3
Helixi108 – 123Combined sources16
Helixi126 – 134Combined sources9
Beta strandi143 – 146Combined sources4
Beta strandi155 – 159Combined sources5
Helixi163 – 172Combined sources10
Helixi173 – 175Combined sources3
Beta strandi179 – 186Combined sources8
Helixi189 – 193Combined sources5
Turni194 – 196Combined sources3
Beta strandi197 – 199Combined sources3
Helixi202 – 208Combined sources7
Beta strandi210 – 214Combined sources5
Turni220 – 224Combined sources5
Helixi228 – 233Combined sources6
Beta strandi239 – 242Combined sources4
Helixi246 – 248Combined sources3
Helixi251 – 259Combined sources9
Beta strandi262 – 269Combined sources8
Beta strandi272 – 275Combined sources4
Helixi281 – 284Combined sources4
Beta strandi288 – 290Combined sources3
Helixi299 – 318Combined sources20

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2GCGX-ray2.20A/B/C/D1-328[»]
2H1SX-ray2.45A/B/C/D2-328[»]
2Q50X-ray2.45A/B/C/D2-328[»]
2WWRX-ray2.82A/B/C/D1-328[»]
ProteinModelPortaliQ9UBQ7.
SMRiQ9UBQ7.
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiQ9UBQ7.

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni83 – 84Substrate binding1 Publication2
Regioni293 – 296Substrate binding1 Publication4

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG0069. Eukaryota.
COG1052. LUCA.
GeneTreeiENSGT00510000046913.
HOGENOMiHOG000136702.
HOVERGENiHBG051838.
InParanoidiQ9UBQ7.
KOiK00049.
OMAiHRTRNTM.
PhylomeDBiQ9UBQ7.
TreeFamiTF324791.

Family and domain databases

Gene3Di3.40.50.720. 2 hits.
InterProiIPR006139. D-isomer_2_OHA_DH_cat_dom.
IPR029753. D-isomer_DH_CS.
IPR006140. D-isomer_DH_NAD-bd.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
PfamiPF00389. 2-Hacid_dh. 1 hit.
PF02826. 2-Hacid_dh_C. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.
PROSITEiPS00671. D_2_HYDROXYACID_DH_3. 1 hit.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9UBQ7-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MRPVRLMKVF VTRRIPAEGR VALARAADCE VEQWDSDEPI PAKELERGVA
60 70 80 90 100
GAHGLLCLLS DHVDKRILDA AGANLKVIST MSVGIDHLAL DEIKKRGIRV
110 120 130 140 150
GYTPDVLTDT TAELAVSLLL TTCRRLPEAI EEVKNGGWTS WKPLWLCGYG
160 170 180 190 200
LTQSTVGIIG LGRIGQAIAR RLKPFGVQRF LYTGRQPRPE EAAEFQAEFV
210 220 230 240 250
STPELAAQSD FIVVACSLTP ATEGLCNKDF FQKMKETAVF INISRGDVVN
260 270 280 290 300
QDDLYQALAS GKIAAAGLDV TSPEPLPTNH PLLTLKNCVI LPHIGSATHR
310 320
TRNTMSLLAA NNLLAGLRGE PMPSELKL
Length:328
Mass (Da):35,668
Last modified:May 1, 2000 - v1
Checksum:i68A0E311AA4E5650
GO
Isoform 2 (identifier: Q9UBQ7-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-21: MRPVRLMKVFVTRRIPAEGRV → MLGGVPTLCGTGNETWTLLAL
     22-164: Missing.
     246-328: GDVVNQDDLY...GEPMPSELKL → YPRATLPSKP...GGQTLKLTWS

Note: No experimental confirmation available.
Show »
Length:252
Mass (Da):27,843
Checksum:i75454F0946FC8BE9
GO

Sequence cautioni

The sequence AAD54066 differs from that shown. Reason: Frameshift at positions 109 and 137.Curated
The sequence AAG39286 differs from that shown. Reason: Frameshift at position 237.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_032762170R → Q.Corresponds to variant rs12002324dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0570161 – 21MRPVR…AEGRV → MLGGVPTLCGTGNETWTLLA L in isoform 2. 1 PublicationAdd BLAST21
Alternative sequenceiVSP_05701722 – 164Missing in isoform 2. 1 PublicationAdd BLAST143
Alternative sequenceiVSP_057018246 – 328GDVVN…SELKL → YPRATLPSKPGEEPSPLLPS GDFLPRGLLVRPQAELAGFH KPNNQLRNSWEYTRPPYREE EPSEWAWPVCFSAVAPTRRG LAHSSVASGSVPREPLQAHY PPPQRAGLEDLKGPLEAASH TAEPGFVWLWFSDTLNLMLL GGQTLKLTWS in isoform 2. 1 PublicationAdd BLAST83

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF134895 mRNA. Translation: AAF00111.1.
AF146018 mRNA. Translation: AAD45886.1.
AF146689 Genomic DNA. Translation: AAD46517.1.
AF113215 mRNA. Translation: AAG39286.1. Frameshift.
AK026287 mRNA. Translation: BAB15430.1.
AK315690 mRNA. Translation: BAG38053.1.
AL158155 Genomic DNA. Translation: CAI13848.1.
CH471071 Genomic DNA. Translation: EAW58284.1.
CH471071 Genomic DNA. Translation: EAW58285.1.
BC000605 mRNA. Translation: AAH00605.1.
AF113251 mRNA. Translation: AAD54066.1. Frameshift.
CCDSiCCDS6609.1. [Q9UBQ7-1]
PIRiJC7190.
RefSeqiNP_036335.1. NM_012203.1. [Q9UBQ7-1]
UniGeneiHs.155742.

Genome annotation databases

EnsembliENST00000318158; ENSP00000313432; ENSG00000137106. [Q9UBQ7-1]
ENST00000494290; ENSP00000432021; ENSG00000137106. [Q9UBQ7-2]
GeneIDi9380.
KEGGihsa:9380.
UCSCiuc003zzu.2. human. [Q9UBQ7-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF134895 mRNA. Translation: AAF00111.1.
AF146018 mRNA. Translation: AAD45886.1.
AF146689 Genomic DNA. Translation: AAD46517.1.
AF113215 mRNA. Translation: AAG39286.1. Frameshift.
AK026287 mRNA. Translation: BAB15430.1.
AK315690 mRNA. Translation: BAG38053.1.
AL158155 Genomic DNA. Translation: CAI13848.1.
CH471071 Genomic DNA. Translation: EAW58284.1.
CH471071 Genomic DNA. Translation: EAW58285.1.
BC000605 mRNA. Translation: AAH00605.1.
AF113251 mRNA. Translation: AAD54066.1. Frameshift.
CCDSiCCDS6609.1. [Q9UBQ7-1]
PIRiJC7190.
RefSeqiNP_036335.1. NM_012203.1. [Q9UBQ7-1]
UniGeneiHs.155742.

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2GCGX-ray2.20A/B/C/D1-328[»]
2H1SX-ray2.45A/B/C/D2-328[»]
2Q50X-ray2.45A/B/C/D2-328[»]
2WWRX-ray2.82A/B/C/D1-328[»]
ProteinModelPortaliQ9UBQ7.
SMRiQ9UBQ7.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi114781. 32 interactors.
IntActiQ9UBQ7. 5 interactors.
MINTiMINT-5005010.
STRINGi9606.ENSP00000313432.

PTM databases

iPTMnetiQ9UBQ7.
PhosphoSitePlusiQ9UBQ7.
SwissPalmiQ9UBQ7.

Polymorphism and mutation databases

BioMutaiGRHPR.
DMDMi47116943.

2D gel databases

REPRODUCTION-2DPAGEIPI00037448.
UCD-2DPAGEQ9UBQ7.

Proteomic databases

EPDiQ9UBQ7.
PaxDbiQ9UBQ7.
PeptideAtlasiQ9UBQ7.
PRIDEiQ9UBQ7.

Protocols and materials databases

DNASUi9380.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000318158; ENSP00000313432; ENSG00000137106. [Q9UBQ7-1]
ENST00000494290; ENSP00000432021; ENSG00000137106. [Q9UBQ7-2]
GeneIDi9380.
KEGGihsa:9380.
UCSCiuc003zzu.2. human. [Q9UBQ7-1]

Organism-specific databases

CTDi9380.
DisGeNETi9380.
GeneCardsiGRHPR.
GeneReviewsiGRHPR.
HGNCiHGNC:4570. GRHPR.
HPAiHPA022971.
MalaCardsiGRHPR.
MIMi260000. phenotype.
604296. gene.
neXtProtiNX_Q9UBQ7.
OpenTargetsiENSG00000137106.
Orphaneti93599. Primary hyperoxaluria type 2.
PharmGKBiPA28965.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG0069. Eukaryota.
COG1052. LUCA.
GeneTreeiENSGT00510000046913.
HOGENOMiHOG000136702.
HOVERGENiHBG051838.
InParanoidiQ9UBQ7.
KOiK00049.
OMAiHRTRNTM.
PhylomeDBiQ9UBQ7.
TreeFamiTF324791.

Enzyme and pathway databases

BioCyciZFISH:HS06275-MONOMER.
ReactomeiR-HSA-389661. Glyoxylate metabolism and glycine degradation.
SABIO-RKQ9UBQ7.

Miscellaneous databases

ChiTaRSiGRHPR. human.
EvolutionaryTraceiQ9UBQ7.
GeneWikiiGRHPR.
GenomeRNAii9380.
PROiQ9UBQ7.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000137106.
CleanExiHS_GRHPR.
ExpressionAtlasiQ9UBQ7. baseline and differential.
GenevisibleiQ9UBQ7. HS.

Family and domain databases

Gene3Di3.40.50.720. 2 hits.
InterProiIPR006139. D-isomer_2_OHA_DH_cat_dom.
IPR029753. D-isomer_DH_CS.
IPR006140. D-isomer_DH_NAD-bd.
IPR016040. NAD(P)-bd_dom.
[Graphical view]
PfamiPF00389. 2-Hacid_dh. 1 hit.
PF02826. 2-Hacid_dh_C. 1 hit.
[Graphical view]
SUPFAMiSSF51735. SSF51735. 1 hit.
PROSITEiPS00671. D_2_HYDROXYACID_DH_3. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiGRHPR_HUMAN
AccessioniPrimary (citable) accession number: Q9UBQ7
Secondary accession number(s): Q5T945
, Q9H3E9, Q9H636, Q9UKX1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 10, 2004
Last sequence update: May 1, 2000
Last modified: November 2, 2016
This is version 155 of the entry and version 1 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 9
    Human chromosome 9: 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.