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Protein

Trans-3-hydroxy-L-proline dehydratase

Gene

L3HYPDH

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

Functioni

Catalyzes the dehydration of trans-3-hydroxy-L-proline to Delta(1)-pyrroline-2-carboxylate (Pyr2C). May be required to degrade trans-3-hydroxy-L-proline from the diet and originating from the degradation of proteins such as collagen-IV that contain it.1 Publication

Catalytic activityi

Trans-3-hydroxy-L-proline = 1-pyrroline 2-carboxylate + H2O.2 Publications

Kineticsi

  1. KM=7.23 mM for trans-3-hydroxy-L-proline1 Publication
  1. Vmax=39.5 µmol/min/mg enzyme1 Publication

pH dependencei

Optimum pH is 8.0.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei104Proton acceptorBy similarity1
Binding sitei269SubstrateBy similarity1

GO - Molecular functioni

  • hydro-lyase activity Source: UniProtKB
  • trans-L-3-hydroxyproline dehydratase activity Source: UniProtKB-EC

GO - Biological processi

  • metabolic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Lyase

Enzyme and pathway databases

BioCyciZFISH:HS13217-MONOMER.

Names & Taxonomyi

Protein namesi
Recommended name:
Trans-3-hydroxy-L-proline dehydratase2 Publications (EC:4.2.1.772 Publications)
Alternative name(s):
Trans-L-3-hydroxyproline dehydratase
Gene namesi
Name:L3HYPDH
Synonyms:C14orf149
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 14

Organism-specific databases

HGNCiHGNC:20488. L3HYPDH.

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi273T → C: Regains racemase activity, catalyzing the conversion of trans-3-hydroxy-L-proline to cis-3-hydroxy-D-proline. Also catalyzes racemization of L-proline to D-proline, albeit at a very low level. Has lost its original dehydratase activity. 1 Publication1

Organism-specific databases

DisGeNETi112849.
OpenTargetsiENSG00000126790.
PharmGKBiPA134961537.

Chemistry databases

DrugBankiDB00172. L-Proline.

Polymorphism and mutation databases

BioMutaiL3HYPDH.
DMDMi296452868.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002889491 – 354Trans-3-hydroxy-L-proline dehydrataseAdd BLAST354

Proteomic databases

EPDiQ96EM0.
MaxQBiQ96EM0.
PaxDbiQ96EM0.
PeptideAtlasiQ96EM0.
PRIDEiQ96EM0.

PTM databases

iPTMnetiQ96EM0.
PhosphoSitePlusiQ96EM0.

Expressioni

Tissue specificityi

Ubiquitously expressed.1 Publication

Gene expression databases

BgeeiENSG00000126790.
CleanExiHS_C14orf149.
ExpressionAtlasiQ96EM0. baseline and differential.
GenevisibleiQ96EM0. HS.

Organism-specific databases

HPAiHPA056694.

Interactioni

Subunit structurei

Homodimer.1 Publication

Protein-protein interaction databases

BioGridi125210. 17 interactors.
STRINGi9606.ENSP00000247194.

Structurei

3D structure databases

ProteinModelPortaliQ96EM0.
SMRiQ96EM0.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni105 – 106Substrate bindingBy similarity2
Regioni274 – 275Substrate bindingBy similarity2

Sequence similaritiesi

Belongs to the proline racemase family.Curated

Phylogenomic databases

eggNOGiENOG410IIC6. Eukaryota.
COG3938. LUCA.
GeneTreeiENSGT00390000002032.
HOGENOMiHOG000084336.
HOVERGENiHBG057443.
InParanoidiQ96EM0.
KOiK18384.
OMAiARMAQWY.
OrthoDBiEOG091G0CI6.
PhylomeDBiQ96EM0.
TreeFamiTF329167.

Family and domain databases

InterProiIPR008794. Pro_racemase_fam.
[Graphical view]
PfamiPF05544. Pro_racemase. 1 hit.
[Graphical view]
PIRSFiPIRSF029792. Pro_racemase. 1 hit.

Sequencei

Sequence statusi: Complete.

Q96EM0-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MESALAVPRL PPHDPGTPVL SVVDMHTGGE PLRIVLAGCP EVSGPTLLAK
60 70 80 90 100
RRYMRQHLDH VRRRLMFEPR GHRDMYGAVL VPSELPDAHL GVLFLHNEGY
110 120 130 140 150
SSMCGHAVLA LGRFALDFGL VPAPPAGTRE ARVNIHCPCG LVTAFVACED
160 170 180 190 200
GRSHGPVRFH SVPAFVLATD LMVDVPGHGK VMVDIAYGGA FYAFVTAEKL
210 220 230 240 250
GLDICSAKTR DLVDAASAVT EAVKAQFKIN HPDSEDLAFL YGTILTDGKD
260 270 280 290 300
AYTKEPTTNI CVFADEQVDR SPTGSGVTAR IALQYHKGLL ELNQMRAFKS
310 320 330 340 350
SATGSVFTGK AVREAKCGDF KAVIVEVSGQ AHYTGTASFI IEDDDPLRDG

FLLK
Length:354
Mass (Da):38,138
Last modified:May 18, 2010 - v2
Checksum:iD279EE0B7C679969
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti9R → W in AAH12131 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_03254042V → A.Corresponds to variant rs17096291dbSNPEnsembl.1
Natural variantiVAR_062192125P → S.Corresponds to variant rs35622288dbSNPEnsembl.1
Natural variantiVAR_032541315A → V.Corresponds to variant rs1046701dbSNPEnsembl.1
Natural variantiVAR_032542341I → V.Corresponds to variant rs8660dbSNPEnsembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK058165 mRNA. Translation: BAB71696.1.
AL159140 Genomic DNA. No translation available.
BC012131 mRNA. Translation: AAH12131.1.
CCDSiCCDS9739.1.
RefSeqiNP_653182.1. NM_144581.1.
UniGeneiHs.729061.

Genome annotation databases

EnsembliENST00000247194; ENSP00000247194; ENSG00000126790.
GeneIDi112849.
KEGGihsa:112849.
UCSCiuc001xee.2. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK058165 mRNA. Translation: BAB71696.1.
AL159140 Genomic DNA. No translation available.
BC012131 mRNA. Translation: AAH12131.1.
CCDSiCCDS9739.1.
RefSeqiNP_653182.1. NM_144581.1.
UniGeneiHs.729061.

3D structure databases

ProteinModelPortaliQ96EM0.
SMRiQ96EM0.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi125210. 17 interactors.
STRINGi9606.ENSP00000247194.

Chemistry databases

DrugBankiDB00172. L-Proline.

PTM databases

iPTMnetiQ96EM0.
PhosphoSitePlusiQ96EM0.

Polymorphism and mutation databases

BioMutaiL3HYPDH.
DMDMi296452868.

Proteomic databases

EPDiQ96EM0.
MaxQBiQ96EM0.
PaxDbiQ96EM0.
PeptideAtlasiQ96EM0.
PRIDEiQ96EM0.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000247194; ENSP00000247194; ENSG00000126790.
GeneIDi112849.
KEGGihsa:112849.
UCSCiuc001xee.2. human.

Organism-specific databases

CTDi112849.
DisGeNETi112849.
GeneCardsiL3HYPDH.
HGNCiHGNC:20488. L3HYPDH.
HPAiHPA056694.
MIMi614811. gene.
neXtProtiNX_Q96EM0.
OpenTargetsiENSG00000126790.
PharmGKBiPA134961537.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiENOG410IIC6. Eukaryota.
COG3938. LUCA.
GeneTreeiENSGT00390000002032.
HOGENOMiHOG000084336.
HOVERGENiHBG057443.
InParanoidiQ96EM0.
KOiK18384.
OMAiARMAQWY.
OrthoDBiEOG091G0CI6.
PhylomeDBiQ96EM0.
TreeFamiTF329167.

Enzyme and pathway databases

BioCyciZFISH:HS13217-MONOMER.

Miscellaneous databases

GenomeRNAii112849.
PROiQ96EM0.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000126790.
CleanExiHS_C14orf149.
ExpressionAtlasiQ96EM0. baseline and differential.
GenevisibleiQ96EM0. HS.

Family and domain databases

InterProiIPR008794. Pro_racemase_fam.
[Graphical view]
PfamiPF05544. Pro_racemase. 1 hit.
[Graphical view]
PIRSFiPIRSF029792. Pro_racemase. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiT3HPD_HUMAN
AccessioniPrimary (citable) accession number: Q96EM0
Secondary accession number(s): Q96LJ5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 29, 2007
Last sequence update: May 18, 2010
Last modified: November 2, 2016
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

Miscellaneous

In contrast to the T.cruzi proline racemase enzyme, lacks the conserved Cys at position 273 which is replaced by a Thr residue, transforming the racemase activity into dehydratase activity.1 Publication

Keywords - Technical termi

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. 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.