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Protein

Glyoxylate reductase/hydroxypyruvate reductase

Gene

GRHPR

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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

  • cellular nitrogen compound metabolic process Source: Reactome
  • dicarboxylic acid metabolic process Source: Ensembl
  • excretion Source: UniProtKB
  • glyoxylate metabolic process Source: UniProtKB
  • oxidation-reduction process Source: UniProtKB
  • protein complex oligomerization Source: UniProtKB

Keywordsi

Molecular functionOxidoreductase
LigandNADP

Enzyme and pathway databases

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

EuPathDBiHostDB:ENSG00000137106.17
HGNCiHGNC:4570 GRHPR
MIMi604296 gene
neXtProtiNX_Q9UBQ7

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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
GeneReviewsiGRHPR
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
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
OMAiKWIAHNG
PhylomeDBiQ9UBQ7
TreeFamiTF324791

Family and domain databases

InterProiView protein in InterPro
IPR006139 D-isomer_2_OHA_DH_cat_dom
IPR029753 D-isomer_DH_CS
IPR006140 D-isomer_DH_NAD-bd
IPR036291 NAD(P)-bd_dom_sf
PfamiView protein in Pfam
PF00389 2-Hacid_dh, 1 hit
PF02826 2-Hacid_dh_C, 1 hit
SUPFAMiSSF51735 SSF51735, 1 hit
PROSITEiView protein in PROSITE
PS00671 D_2_HYDROXYACID_DH_3, 1 hit

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 dbSNP:rs12002324Ensembl.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 No translation available.
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

Similar proteinsi

Entry informationi

Entry nameiGRHPR_HUMAN
AccessioniPrimary (citable) accession number: Q9UBQ7
Secondary accession number(s): Q5T945
, Q9H3E9, Q9H636, Q9UKX1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 10, 2004
Last sequence update: May 1, 2000
Last modified: May 23, 2018
This is version 166 of the entry and version 1 of the sequence. See 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

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