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Protein

Peripherin

Gene

PRPH

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

Functioni

Class-III neuronal intermediate filament protein.

GO - Molecular functioni

  • structural molecule activity Source: ProtInc

Names & Taxonomyi

Protein namesi
Recommended name:
Peripherin
Alternative name(s):
Neurofilament 4
Gene namesi
Name:PRPH
Synonyms:NEF4, PRPH1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 12

Organism-specific databases

EuPathDBiHostDB:ENSG00000135406.13.
HGNCiHGNC:9461. PRPH.

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

Intermediate filament

Pathology & Biotechi

Organism-specific databases

DisGeNETi5630.
MalaCardsiPRPH.
OpenTargetsiENSG00000135406.
Orphaneti803. Amyotrophic lateral sclerosis.
PharmGKBiPA33816.

Polymorphism and mutation databases

BioMutaiPRPH.
DMDMi118585871.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000637791 – 470PeripherinAdd BLAST470

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei13PhosphoserineBy similarity1
Modified residuei14PhosphoserineBy similarity1
Modified residuei16PhosphoserineBy similarity1
Modified residuei17Nitrated tyrosineBy similarity1
Modified residuei19Omega-N-methylarginineBy similarity1
Modified residuei20PhosphothreonineBy similarity1
Modified residuei28PhosphoserineBy similarity1
Modified residuei33PhosphoserineBy similarity1
Modified residuei36PhosphoserineBy similarity1
Modified residuei41PhosphoserineBy similarity1
Modified residuei43PhosphoserineBy similarity1
Modified residuei50PhosphoserineBy similarity1
Modified residuei53PhosphoserineBy similarity1
Modified residuei81PhosphoserineBy similarity1
Cross-linki98Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)By similarity
Modified residuei114N6-acetyllysine; alternateBy similarity1
Modified residuei114N6-succinyllysine; alternateBy similarity1
Cross-linki114Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateBy similarity
Modified residuei217N6-acetyllysine; alternateBy similarity1
Cross-linki217Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateBy similarity
Modified residuei220PhosphoserineBy similarity1
Modified residuei229N6-acetyllysineBy similarity1
Cross-linki258Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)By similarity
Modified residuei290N6-acetyllysine; alternateBy similarity1
Modified residuei290N6-succinyllysine; alternateBy similarity1
Cross-linki290Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateBy similarity
Modified residuei295PhosphoserineBy similarity1
Cross-linki309Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)By similarity
Modified residuei321PhosphoserineBy similarity1
Modified residuei369N6-acetyllysine; alternateBy similarity1
Cross-linki369Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateBy similarity
Modified residuei379Nitrated tyrosineBy similarity1
Modified residuei405PhosphoserineBy similarity1
Modified residuei408PhosphoserineBy similarity1
Modified residuei416PhosphoserineBy similarity1
Modified residuei422PhosphothreonineBy similarity1
Modified residuei434PhosphoserineBy similarity1
Modified residuei441N6-acetyllysine; alternateBy similarity1
Modified residuei441N6-succinyllysine; alternateBy similarity1
Cross-linki441Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO1); alternateBy similarity
Cross-linki441Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2); alternateBy similarity
Modified residuei442PhosphothreonineBy similarity1

Keywords - PTMi

Acetylation, Isopeptide bond, Methylation, Nitration, Phosphoprotein, Ubl conjugation

Proteomic databases

EPDiP41219.
MaxQBiP41219.
PaxDbiP41219.
PeptideAtlasiP41219.
PRIDEiP41219.

PTM databases

iPTMnetiP41219.
PhosphoSitePlusiP41219.
SwissPalmiP41219.

Expressioni

Gene expression databases

BgeeiENSG00000135406.
CleanExiHS_PRPH.
ExpressionAtlasiP41219. baseline and differential.
GenevisibleiP41219. HS.

Organism-specific databases

HPAiCAB002437.
HPA039277.
HPA063887.

Interactioni

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi111614. 17 interactors.
IntActiP41219. 39 interactors.
MINTiMINT-1482695.
STRINGi9606.ENSP00000257860.

Structurei

3D structure databases

ProteinModelPortaliP41219.
SMRiP41219.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini97 – 407IF rodPROSITE-ProRule annotationAdd BLAST311

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni1 – 99HeadAdd BLAST99
Regioni100 – 132Coil 1AAdd BLAST33
Regioni133 – 143Linker 1Add BLAST11
Regioni144 – 239Coil 1BAdd BLAST96
Regioni240 – 262Linker 2Add BLAST23
Regioni263 – 405Coil 2Add BLAST143
Regioni406 – 470TailAdd BLAST65

Sequence similaritiesi

Belongs to the intermediate filament family.PROSITE-ProRule annotation

Keywords - Domaini

Coiled coil

Phylogenomic databases

eggNOGiENOG410IE39. Eukaryota.
ENOG410YCMG. LUCA.
GeneTreeiENSGT00830000128228.
HOGENOMiHOG000230977.
HOVERGENiHBG013015.
InParanoidiP41219.
KOiK07607.
OMAiEIQDVQV.
OrthoDBiEOG091G12MK.
PhylomeDBiP41219.
TreeFamiTF330122.

Family and domain databases

InterProiView protein in InterPro
IPR001664. IF.
IPR006821. Intermed_filament_DNA-bd.
IPR018039. Intermediate_filament_CS.
IPR002957. Keratin_I.
IPR027700. PRPH/Plasticin.
PANTHERiPTHR23239. PTHR23239. 1 hit.
PTHR23239:SF29. PTHR23239:SF29. 1 hit.
PfamiView protein in Pfam
PF00038. Filament. 1 hit.
PF04732. Filament_head. 1 hit.
PRINTSiPR01248. TYPE1KERATIN.
SMARTiView protein in SMART
SM01391. Filament. 1 hit.
PROSITEiView protein in PROSITE
PS00226. IF_ROD_1. 1 hit.
PS51842. IF_ROD_2. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

Isoform 1 (identifier: P41219-1) [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
MSHHPSGLRA GFSSTSYRRT FGPPPSLSPG AFSYSSSSRF SSSRLLGSAS
60 70 80 90 100
PSSSVRLGSF RSPRAGAGAL LRLPSERLDF SMAEALNQEF LATRSNEKQE
110 120 130 140 150
LQELNDRFAN FIEKVRFLEQ QNAALRGELS QARGQEPARA DQLCQQELRE
160 170 180 190 200
LRRELELLGR ERDRVQVERD GLAEDLAALK QRLEEETRKR EDAEHNLVLF
210 220 230 240 250
RKDVDDATLS RLELERKIES LMDEIEFLKK LHEEELRDLQ VSVESQQVQQ
260 270 280 290 300
VEVEATVKPE LTAALRDIRA QYESIAAKNL QEAEEWYKSK YADLSDAANR
310 320 330 340 350
NHEALRQAKQ EMNESRRQIQ SLTCEVDGLR GTNEALLRQL RELEEQFALE
360 370 380 390 400
AGGYQAGAAR LEEELRQLKE EMARHLREYQ ELLNVKMALD IEIATYRKLL
410 420 430 440 450
EGEESRISVP VHSFASLNIK TTVPEVEPPQ DSHSRKTVLI KTIETRNGEV
460 470
VTESQKEQRS ELDKSSAHSY
Length:470
Mass (Da):53,651
Last modified:October 31, 2006 - v2
Checksum:i4ABEAB96088719D6
GO
Isoform 2 (identifier: P41219-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     449-449: E → EQ

Note: No experimental confirmation available. Gene prediction based on similarity to orthologs.
Show »
Length:471
Mass (Da):53,779
Checksum:i8138A4773E53D86A
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti68G → R in AAA60190 (PubMed:7806235).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_021159449E → EQ in isoform 2. Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L14565 Genomic DNA. Translation: AAA60190.1.
AC125611 Genomic DNA. No translation available.
BC032703 mRNA. Translation: AAH32703.1.
CCDSiCCDS8783.1. [P41219-1]
PIRiA55185.
RefSeqiNP_006253.2. NM_006262.3. [P41219-1]
XP_005269082.1. XM_005269025.1. [P41219-2]
UniGeneiHs.37044.

Genome annotation databases

EnsembliENST00000257860; ENSP00000257860; ENSG00000135406. [P41219-1]
GeneIDi5630.
KEGGihsa:5630.
UCSCiuc001rtu.4. human. [P41219-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiPERI_HUMAN
AccessioniPrimary (citable) accession number: P41219
Secondary accession number(s): Q8N577
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1995
Last sequence update: October 31, 2006
Last modified: October 25, 2017
This is version 155 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

Documents

  1. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  2. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  3. SIMILARITY comments
    Index of protein domains and families