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Protein

Peptidyl-prolyl cis-trans isomerase H

Gene

PPIH

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

Functioni

PPIases accelerate the folding of proteins. It catalyzes the cis-trans isomerization of proline imidic peptide bonds in oligopeptides. Participates in pre-mRNA splicing. May play a role in the assembly of the U4/U5/U6 tri-snRNP complex, one of the building blocks of the spliceosome. May act as a chaperone.3 Publications

Caution

Catalytic activityi

Peptidylproline (omega=180) = peptidylproline (omega=0).

Enzyme regulationi

Inhibited by cyclosporin A.

GO - Molecular functioni

  • cyclosporin A binding Source: ProtInc
  • peptidyl-prolyl cis-trans isomerase activity Source: BHF-UCL
  • ribonucleoprotein complex binding Source: BHF-UCL

GO - Biological processi

  • mRNA splicing, via spliceosome Source: Reactome
  • positive regulation of viral genome replication Source: UniProtKB
  • protein-containing complex assembly Source: ProtInc
  • protein folding Source: ProtInc

Keywordsi

Molecular functionChaperone, Isomerase, Rotamase
Biological processmRNA processing, mRNA splicing

Enzyme and pathway databases

BRENDAi5.2.1.8 2681
ReactomeiR-HSA-72163 mRNA Splicing - Major Pathway

Names & Taxonomyi

Protein namesi
Recommended name:
Peptidyl-prolyl cis-trans isomerase H (EC:5.2.1.8)
Short name:
PPIase H
Alternative name(s):
Rotamase H
Small nuclear ribonucleoprotein particle-specific cyclophilin H
Short name:
CypH
U-snRNP-associated cyclophilin SnuCyp-20
Short name:
USA-CYP
Gene namesi
Name:PPIH
Synonyms:CYP20, CYPH
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000171960.10
HGNCiHGNC:14651 PPIH
MIMi606095 gene
neXtProtiNX_O43447

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

Keywords - Cellular componenti

Cytoplasm, Nucleus, Spliceosome

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi133W → F: Abolishes inhibition by cyclosporin A. 1 Publication1

Organism-specific databases

OpenTargetsiENSG00000171960
PharmGKBiPA33586

Chemistry databases

DrugBankiDB00172 L-Proline

Polymorphism and mutation databases

BioMutaiPPIH

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00000641622 – 177Peptidyl-prolyl cis-trans isomerase HAdd BLAST176

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiO43447
PaxDbiO43447
PeptideAtlasiO43447
PRIDEiO43447

PTM databases

iPTMnetiO43447
PhosphoSitePlusiO43447

Expressioni

Gene expression databases

BgeeiENSG00000171960
CleanExiHS_PPIH
ExpressionAtlasiO43447 baseline and differential
GenevisibleiO43447 HS

Organism-specific databases

HPAiHPA059019

Interactioni

Subunit structurei

Interacts directly with PRPF4. Part of a heteromeric complex containing PPIH, PRPF3 and PRPF4 that is stable in the absence of RNA. Component of the U4/U6-U5 tri-snRNP complex composed of the U4, U6 and U5 snRNAs and at least PRPF3, PRPF4, PRPF6, PRPF8, PRPF31, SNRNP200, TXNL4A, SNRNP40, DDX23, CD2BP2, PPIH, SNU13, EFTUD2, SART1 and USP39. Heterodimer with PRPF18.5 Publications

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi11572834 interactors.
CORUMiO43447
IntActiO43447 29 interactors.
MINTiO43447
STRINGi9606.ENSP00000306614

Chemistry databases

BindingDBiO43447

Structurei

Secondary structure

1177
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Beta strandi12 – 19Combined sources8
Beta strandi22 – 31Combined sources10
Turni33 – 35Combined sources3
Helixi37 – 48Combined sources12
Beta strandi67 – 69Combined sources3
Turni70 – 72Combined sources3
Beta strandi73 – 76Combined sources4
Turni79 – 81Combined sources3
Beta strandi82 – 84Combined sources3
Beta strandi109 – 112Combined sources4
Beta strandi124 – 129Combined sources6
Helixi132 – 134Combined sources3
Turni135 – 137Combined sources3
Beta strandi140 – 146Combined sources7
Helixi148 – 155Combined sources8
Helixi161 – 163Combined sources3
Beta strandi165 – 167Combined sources3
Beta strandi169 – 176Combined sources8

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1MZWX-ray2.00A1-177[»]
1QOIX-ray2.00A1-177[»]
5O9Zelectron microscopy4.50M1-177[»]
ProteinModelPortaliO43447
SMRiO43447
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiO43447

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini14 – 176PPIase cyclophilin-typePROSITE-ProRule annotationAdd BLAST163

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG0546 Eukaryota
COG0652 LUCA
GeneTreeiENSGT00760000119072
HOGENOMiHOG000065981
HOVERGENiHBG001065
InParanoidiO43447
KOiK09567
OMAiVPFHRVM
OrthoDBiEOG091G0BGL
PhylomeDBiO43447
TreeFamiTF312958

Family and domain databases

Gene3Di2.40.100.101 hit
InterProiView protein in InterPro
IPR029000 Cyclophilin-like_dom_sf
IPR024936 Cyclophilin-type_PPIase
IPR020892 Cyclophilin-type_PPIase_CS
IPR002130 Cyclophilin-type_PPIase_dom
PANTHERiPTHR11071 PTHR11071, 1 hit
PfamiView protein in Pfam
PF00160 Pro_isomerase, 1 hit
PIRSFiPIRSF001467 Peptidylpro_ismrse, 1 hit
PRINTSiPR00153 CSAPPISMRASE
SUPFAMiSSF50891 SSF50891, 1 hit
PROSITEiView protein in PROSITE
PS00170 CSA_PPIASE_1, 1 hit
PS50072 CSA_PPIASE_2, 1 hit

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

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

Isoform 1 (identifier: O43447-1) [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
MAVANSSPVN PVVFFDVSIG GQEVGRMKIE LFADVVPKTA ENFRQFCTGE
60 70 80 90 100
FRKDGVPIGY KGSTFHRVIK DFMIQGGDFV NGDGTGVASI YRGPFADENF
110 120 130 140 150
KLRHSAPGLL SMANSGPSTN GCQFFITCSK CDWLDGKHVV FGKIIDGLLV
160 170
MRKIENVPTG PNNKPKLPVV ISQCGEM
Length:177
Mass (Da):19,208
Last modified:June 1, 1998 - v1
Checksum:i566BCE6361E0F339
GO
Isoform 2 (identifier: O43447-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-51: MAVANSSPVNPVVFFDVSIGGQEVGRMKIELFADVVPKTAENFRQFCTGEF → MIAGDSDR

Note: No experimental confirmation available.
Show »
Length:134
Mass (Da):14,492
Checksum:iE55350B73F8A1C3B
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0565871 – 51MAVAN…CTGEF → MIAGDSDR in isoform 2. 1 PublicationAdd BLAST51

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF016371 mRNA Translation: AAC51927.1
AF036331 mRNA Translation: AAC60793.1
AK294288 mRNA Translation: BAH11725.1
AC098484 Genomic DNA No translation available.
CH471059 Genomic DNA Translation: EAX07155.1
BC003412 mRNA Translation: AAH03412.1
CCDSiCCDS469.1 [O43447-1]
CCDS81308.1 [O43447-2]
RefSeqiNP_001317439.1, NM_001330510.1 [O43447-2]
NP_006338.1, NM_006347.3 [O43447-1]
XP_005270419.1, XM_005270362.1 [O43447-1]
XP_016855546.1, XM_017000057.1 [O43447-1]
XP_016855547.1, XM_017000058.1 [O43447-2]
XP_016855548.1, XM_017000059.1 [O43447-2]
UniGeneiHs.256639
Hs.729213

Genome annotation databases

EnsembliENST00000304979; ENSP00000306614; ENSG00000171960 [O43447-1]
ENST00000372550; ENSP00000361630; ENSG00000171960 [O43447-2]
GeneIDi10465
KEGGihsa:10465
UCSCiuc009vwl.3 human [O43447-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiPPIH_HUMAN
AccessioniPrimary (citable) accession number: O43447
Secondary accession number(s): A6NNE7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 26, 2003
Last sequence update: June 1, 1998
Last modified: April 25, 2018
This is version 164 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, Direct protein sequencing, Reference proteome