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UniProtKB - Q9R044 (NPHN_RAT)

Protein

Nephrin

Gene

Nphs1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Seems to play a role in the development or function of the kidney glomerular filtration barrier. Regulates glomerular vascular permeability. May anchor the podocyte slit diaphragm to the actin cytoskeleton. Plays a role in skeletal muscle formation through regulation of myoblast fusion.2 Publications

GO - Molecular functioni

  • alpha-actinin binding Source: RGD
  • protein domain specific binding Source: RGD
  • spectrin binding Source: RGD
View the complete GO annotation on QuickGO ...

GO - Biological processi

  • cell adhesion Source: UniProtKB-KW
  • glomerular filtration Source: UniProtKB
  • muscle organ development Source: UniProtKB-KW
View the complete GO annotation on QuickGO ...

Keywordsi

Molecular functionDevelopmental protein
Biological processCell adhesion, Myogenesis

Names & Taxonomyi

Protein namesi
Recommended name:
Nephrin
Alternative name(s):
Renal glomerulus-specific cell adhesion receptor
Gene namesi
Name:Nphs1
Synonyms:Nphn
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Rattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi620460 Nphs1

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini36 – 1078ExtracellularSequence analysisAdd BLAST1043
Transmembranei1079 – 1099HelicalSequence analysisAdd BLAST21
Topological domaini1100 – 1252CytoplasmicSequence analysisAdd BLAST153

Keywords - Cellular componenti

Cell membrane, Membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 35Sequence analysisAdd BLAST35
ChainiPRO_000001505436 – 1252NephrinAdd BLAST1217

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi54N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi67 ↔ 125PROSITE-ProRule annotation
Disulfide bondi174 ↔ 231PROSITE-ProRule annotation
Disulfide bondi279 ↔ 331PROSITE-ProRule annotation
Glycosylationi370N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi375 ↔ 431PROSITE-ProRule annotation
Modified residuei446PhosphoserineBy similarity1
Disulfide bondi479 ↔ 542PROSITE-ProRule annotation
Glycosylationi561N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi578N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi581 ↔ 637PROSITE-ProRule annotation
Glycosylationi591N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi722N-linked (GlcNAc...) asparagineSequence analysis1
Disulfide bondi775 ↔ 830PROSITE-ProRule annotation
Disulfide bondi877 ↔ 934PROSITE-ProRule annotation
Modified residuei1112PhosphoserineCombined sources1
Modified residuei1115PhosphothreonineCombined sources1
Modified residuei1119PhosphoserineCombined sources1
Modified residuei1204Phosphotyrosine; by FYN1 Publication1

Post-translational modificationi

Phosphorylated at Tyr-1204 by FYN, leading to the recruitment and activation of phospholipase C-gamma-1/PLCG1.1 Publication

Keywords - PTMi

Disulfide bond, Glycoprotein, Phosphoprotein

Proteomic databases

PaxDbiQ9R044
PRIDEiQ9R044

PTM databases

iPTMnetiQ9R044
PhosphoSitePlusiQ9R044

Expressioni

Tissue specificityi

Strongly expressed in the podocytes of kidney glomeruli (at protein level) and at lower levels in the spleen.2 Publications

Inductioni

Following injection with puromycin which induces nephrosis, down-regulated by 40% 3 days post-injection and by 80% at day 10. Also down-regulated by HgCl2 with rapid decrease at day 3.1 Publication

Interactioni

Subunit structurei

Interacts with NPHS2 and with CD2AP (via C-terminal domain). Self-associates (via the Ig-like domains). Also interacts (via the Ig-like domains) with KIRREL1/NEPH1 and KIRREL2; the interaction with KIRREL1 is dependent on KIRREL1 glycosylation. Interacts with KIRREL3 (By similarity). Interacts with MAGI1 (via PDZ 2 and 3 domains) forming a tripartite complex with IGSF5/JAM4. Interacts with DDN; the interaction is direct. Forms a complex with ACTN4, CASK, IQGAP1, MAGI2, SPTAN1 and SPTBN1.By similarity3 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
SirpaP977102EBI-7945021,EBI-7945080

GO - Molecular functioni

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridi249144, 8 interactors
CORUMiQ9R044
IntActiQ9R044, 1 interactor
MINTiQ9R044
STRINGi10116.ENSRNOP00000049922

Structurei

3D structure databases

ProteinModelPortaliQ9R044
SMRiQ9R044
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini39 – 144Ig-like C2-type 1Add BLAST106
Domaini149 – 247Ig-like C2-type 2Add BLAST99
Domaini256 – 347Ig-like C2-type 3Add BLAST92
Domaini354 – 448Ig-like C2-type 4Add BLAST95
Domaini454 – 554Ig-like C2-type 5Add BLAST101
Domaini558 – 649Ig-like C2-type 6Add BLAST92
Domaini754 – 846Ig-like C2-type 7Add BLAST93
Domaini852 – 953Ig-like C2-type 8Add BLAST102
Domaini957 – 1052Fibronectin type-IIIPROSITE-ProRule annotationAdd BLAST96

Sequence similaritiesi

Belongs to the immunoglobulin superfamily.Curated

Keywords - Domaini

Immunoglobulin domain, Repeat, Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3515 Eukaryota
ENOG410XRJN LUCA
HOGENOMiHOG000113844
HOVERGENiHBG031752
InParanoidiQ9R044
PhylomeDBiQ9R044

Family and domain databases

CDDicd00063 FN3, 1 hit
Gene3Di2.60.40.10, 10 hits
InterProiView protein in InterPro
IPR013162 CD80_C2-set
IPR003961 FN3_dom
IPR036116 FN3_sf
IPR007110 Ig-like_dom
IPR036179 Ig-like_dom_sf
IPR013783 Ig-like_fold
IPR003599 Ig_sub
IPR003598 Ig_sub2
PfamiView protein in Pfam
PF08205 C2-set_2, 4 hits
PF00041 fn3, 1 hit
SMARTiView protein in SMART
SM00060 FN3, 1 hit
SM00409 IG, 8 hits
SM00408 IGc2, 7 hits
SUPFAMiSSF48726 SSF48726, 9 hits
SSF49265 SSF49265, 1 hit
PROSITEiView protein in PROSITE
PS50853 FN3, 1 hit
PS50835 IG_LIKE, 7 hits

Sequences (3+)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket
Note: Experimental confirmation may be lacking for some isoforms.

This entry has 3 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9R044-1) [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
MGAKRVTVRG ARTSPIHRMS SLTPLLLMGM LTSGLAESPV PTSAPRGFWA 
        60         70         80         90        100
LSENLTAVEG TTVKLWCGVR APGSVVQWAK DGLLLGPNPK MPGFPRYSLE 
       110        120        130        140        150
GDRAKGEFHL LIEACDLSDD AEYECQVGRS ELGPELVSPK VILSILVSPK 
       160        170        180        190        200
VLLLTPEAGS TVTWVAGQEY VVTCVSGDAK PAPDITFIQS GRTILDVSSN 
       210        220        230        240        250
VNEGSEEKLC ITEAEARVIP QSSDNGQLLV CEGSNPALDT PIKASFTMNI 
       260        270        280        290        300
LFPPGPPVID WPGLNEGHVR AGENLELPCT ARGGNPPATL QWLKNGKPVS 
       310        320        330        340        350
TAWGTEHAQA VAHSVLVMTV RPEDHGARLS CQSYNSVSAG TQERSITLQV 
       360        370        380        390        400
TFPPSAITIL GSVSQSENKN VTLCCLTKSS RPRVLLRWWL GGRQLLPTDE 
       410        420        430        440        450
TVMDGLHGGH ISMSNLTFLV RREDNGLPLT CEAFSDAFSK ETFKKSLTLN 
       460        470        480        490        500
VKYPAQKLWI EGPPEGQYIR TGTRVRLVCL AIGGNPDPSL IWFKDSRPVS 
       510        520        530        540        550
EPRQPQEPRR VQLGSVEKSG STFSRELVLI IGPPDNRAKF SCKAGQLSAS 
       560        570        580        590        600
TQLVVQFPPT NLTILANSSA LRPGDALNLT CVSISSNPPV NLSWDKEGER 
       610        620        630        640        650
LEDVAAKPQS APFKGSAASR SVFLRVSSRD HGQRVTCRAH SEALRETVSS 
       660        670        680        690        700
FYRFNVLYPP EFLGEQVRAV TVVEQGQVLL PVSVSANPAP EAFNWTFRGY 
       710        720        730        740        750
RLSPAGGPRH RILSGGALQL WNVTRADDGF YQLHCQNSEG TAEALLKLDV 
       760        770        780        790        800
HYAPTIRALR DPTEVNVGGS VDIVCTVDAN PILPEMFSWE RLGEEEEDLN 
       810        820        830        840        850
LDDMEKVSKG STGRLRIRQA KLSQAGAYQC IVDNGVAPAA RGLVRLVVRF 
       860        870        880        890        900
APQVDQPTPL TKVAAAGDST SSATLHCRAR GVPNIDFTWT KNGVPLDLQD 
       910        920        930        940        950
PRYTEHRYHQ GVVHSSLLTI ANVSAAQDYA LFKCTATNAL GSDHTNIQLV 
       960        970        980        990       1000
SISRPDPPLG LKVVSISPHS VGLEWKPGFD GGLPQRFQIR YEALETPGFL 
      1010       1020       1030       1040       1050
HVDVLPTQAT TFTLTGLKPS TRYRIWLLAS NALGDSGLTD KGIQVSVTTP 
      1060       1070       1080       1090       1100
GPDQAPEDTD HQLPTELPPG PPRLPLLPVL FAVGGLLLLS NASCVGGLLW 
      1110       1120       1130       1140       1150
RRRLRRLAEE ISEKTEAGSE DRIRNEYEES QWTGDRDTRS STVSTAEVDP 
      1160       1170       1180       1190       1200
NYYSMRDFSP QLPPTLEEVL YHQGAEGEDM AFPGHLHDEV ERAYGPPGAW 
      1210       1220       1230       1240       1250
GPLYDEVRMD PYDLRWPEVQ CEDPRGIYDQ VAADMDAVEA SSLPFELRGH 

LV
Length:1,252
Mass (Da):136,281
Last modified:March 8, 2011 - v2
Checksum:i6A3550AB907883EC
GO
Isoform 2 (identifier: Q9R044-2) [UniParc]FASTAAdd to basket
Also known as: Alpha

The sequence of this isoform differs from the canonical sequence as follows:
     1070-1082: Missing.

Show »
Length:1,239
Mass (Da):134,910
Checksum:i8127731D79F1F183
GO
Isoform 3 (identifier: Q9R044-3) [UniParc]FASTAAdd to basket
Also known as: Beta

The sequence of this isoform differs from the canonical sequence as follows:
     1070-1155: Missing.

Show »
Length:1,166
Mass (Da):126,646
Checksum:iC22AF65387FAD4B5
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A140TAG4A0A140TAG4_RAT
Nephrin
Nphs1
1,252Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti19M → T in AAF14884 (PubMed:10792613).Curated1
Sequence conflicti46R → Q in AAF14884 (PubMed:10792613).Curated1
Sequence conflicti1229D → N in AAF12734 (PubMed:10487848).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0026001070 – 1155Missing in isoform 3. 1 PublicationAdd BLAST86
Alternative sequenceiVSP_0025991070 – 1082Missing in isoform 2. 1 PublicationAdd BLAST13

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF172255 mRNA Translation: AAF91086.1
AF161715 mRNA Translation: AAF14884.1
AF125521 mRNA Translation: AAF12734.1
RefSeqiNP_072150.1, NM_022628.1
UniGeneiRn.48745

Genome annotation databases

GeneIDi64563
KEGGirno:64563
UCSCiRGD:620460 rat [Q9R044-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF172255 mRNA Translation: AAF91086.1
AF161715 mRNA Translation: AAF14884.1
AF125521 mRNA Translation: AAF12734.1
RefSeqiNP_072150.1, NM_022628.1
UniGeneiRn.48745

3D structure databases

ProteinModelPortaliQ9R044
SMRiQ9R044
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi249144, 8 interactors
CORUMiQ9R044
IntActiQ9R044, 1 interactor
MINTiQ9R044
STRINGi10116.ENSRNOP00000049922

PTM databases

iPTMnetiQ9R044
PhosphoSitePlusiQ9R044

Proteomic databases

PaxDbiQ9R044
PRIDEiQ9R044

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi64563
KEGGirno:64563
UCSCiRGD:620460 rat [Q9R044-1]

Organism-specific databases

CTDi4868
RGDi620460 Nphs1

Phylogenomic databases

eggNOGiKOG3515 Eukaryota
ENOG410XRJN LUCA
HOGENOMiHOG000113844
HOVERGENiHBG031752
InParanoidiQ9R044
PhylomeDBiQ9R044

Miscellaneous databases

PROiPR:Q9R044

Family and domain databases

CDDicd00063 FN3, 1 hit
Gene3Di2.60.40.10, 10 hits
InterProiView protein in InterPro
IPR013162 CD80_C2-set
IPR003961 FN3_dom
IPR036116 FN3_sf
IPR007110 Ig-like_dom
IPR036179 Ig-like_dom_sf
IPR013783 Ig-like_fold
IPR003599 Ig_sub
IPR003598 Ig_sub2
PfamiView protein in Pfam
PF08205 C2-set_2, 4 hits
PF00041 fn3, 1 hit
SMARTiView protein in SMART
SM00060 FN3, 1 hit
SM00409 IG, 8 hits
SM00408 IGc2, 7 hits
SUPFAMiSSF48726 SSF48726, 9 hits
SSF49265 SSF49265, 1 hit
PROSITEiView protein in PROSITE
PS50853 FN3, 1 hit
PS50835 IG_LIKE, 7 hits
ProtoNetiSearch...

Entry informationi

Entry nameiNPHN_RAT
AccessioniPrimary (citable) accession number: Q9R044
Secondary accession number(s): Q9JIX2, Q9QXX7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 16, 2002
Last sequence update: March 8, 2011
Last modified: June 20, 2018
This is version 150 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
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