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Protein

Trans-3-hydroxy-L-proline dehydratase

Gene

L3HYPDH

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

Functioni

Catalyzes the dehydration of trans-3-hydroxy-L-proline to Delta1-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

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

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

Keywordsi

Molecular functionLyase

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

EuPathDBiHostDB:ENSG00000126790.11
HGNCiHGNC:20488 L3HYPDH
MIMi614811 gene
neXtProtiNX_Q96EM0

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
ProteomicsDBi76425

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, 18 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
OMAiRHAMAIH
OrthoDBiEOG091G0CI6
PhylomeDBiQ96EM0
TreeFamiTF329167

Family and domain databases

InterProiView protein in InterPro
IPR008794 Pro_racemase_fam
PANTHERiPTHR33442 PTHR33442, 1 hit
PfamiView protein in Pfam
PF05544 Pro_racemase, 1 hit
PIRSFiPIRSF029792 Pro_racemase, 1 hit
SFLDiSFLDS00028 Proline_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 dbSNP:rs17096291Ensembl.1
Natural variantiVAR_062192125P → S. Corresponds to variant dbSNP:rs35622288Ensembl.1
Natural variantiVAR_032541315A → V. Corresponds to variant dbSNP:rs1046701Ensembl.1
Natural variantiVAR_032542341I → V. Corresponds to variant dbSNP:rs8660Ensembl.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

Similar proteinsi

Entry informationi

Entry nameiT3HPD_HUMAN
AccessioniPrimary (citable) accession number: Q96EM0
Secondary accession number(s): Q96LJ5
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 29, 2007
Last sequence update: May 18, 2010
Last modified: June 20, 2018
This is version 107 of the entry and version 2 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

Complete proteome, Reference proteome

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