Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

FRAS1-related extracellular matrix protein 2

Gene

Frem2

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Extracellular matrix protein required for maintenance of the integrity of the skin epithelium and for maintenance of renal epithelia. May be required for epidermal adhesion.1 Publication

GO - Molecular functioni

GO - Biological processi

  • cell adhesion Source: UniProtKB-KW
  • cell communication Source: InterPro
  • embryonic digit morphogenesis Source: MGI
  • eye development Source: MGI
  • heart development Source: MGI
  • inner ear development Source: MGI
  • morphogenesis of an epithelium Source: MGI

Keywordsi

Molecular functionDevelopmental protein
Biological processCell adhesion
LigandCalcium, Metal-binding

Names & Taxonomyi

Protein namesi
Recommended name:
FRAS1-related extracellular matrix protein 2
Alternative name(s):
ECM3 homolog
NV domain-containing protein 1
Gene namesi
Name:Frem2
Synonyms:Nv1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 3

Organism-specific databases

MGIiMGI:2444465 Frem2

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 domaini40 – 3105ExtracellularSequence analysisAdd BLAST3066
Transmembranei3106 – 3126HelicalSequence analysisAdd BLAST21
Topological domaini3127 – 3160CytoplasmicSequence analysisAdd BLAST34

Keywords - Cellular componenti

Cell membrane, Membrane

Pathology & Biotechi

Disruption phenotypei

Mice display cryptophthalmos, syndactyly and renal defects. Frem2 corresponds to the X-ray irradiated-induced allele 'myelencephalic blebs' (my).1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 39Sequence analysisAdd BLAST39
ChainiPRO_000001012540 – 3160FRAS1-related extracellular matrix protein 2Add BLAST3121

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi351N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi1233N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi1358N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi1573N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi1730N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Glycoprotein

Proteomic databases

EPDiQ6NVD0
PaxDbiQ6NVD0
PRIDEiQ6NVD0

PTM databases

iPTMnetiQ6NVD0
PhosphoSitePlusiQ6NVD0

Expressioni

Tissue specificityi

First expressed from E10 in the mesodermal core of the branchial arches, developing lens, otic vesicle and limb apical ectodermal ridge. Later, it is expressed in the vibrissae and vibrissae pad, eyelids ear and pelage follicles and, at low levels, in the epidermis. Also expressed in caudal somites and, in later embryos, in facial, limb and intercostal muscles. In contrast to Frem1, it is not expressed in the developing mammary glands or in the caecum. Restricted to the epithelia in a pattern complementary to that of Frem1 (which is generally expressed in the dermis and mesenchyme). In the developing kidney, it is expressed At in the mesonephric and metanephric epithelia at E11.5, with a highest expression at the tips of the developing ureteric buds. At E12.5 and E13.5, it is still expressed throughout the epithelial components of the kidney, including epithelia fated to form nephrons, which are induced by the ureter tips to differentiate from the mesenchymal condensations that surround them.1 Publication

Gene expression databases

BgeeiENSMUSG00000037016
CleanExiMM_FREM2
GenevisibleiQ6NVD0 MM

Interactioni

Binary interactionsi

WithEntry#Exp.IntActNotes
Fras1Q80T142EBI-15594269,EBI-15594303

Protein-protein interaction databases

DIPiDIP-61241N
IntActiQ6NVD0, 2 interactors
STRINGi10090.ENSMUSP00000088670

Structurei

3D structure databases

ProteinModelPortaliQ6NVD0
SMRiQ6NVD0
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Repeati312 – 406CSPG 1PROSITE-ProRule annotationAdd BLAST95
Repeati431 – 530CSPG 2PROSITE-ProRule annotationAdd BLAST100
Repeati553 – 664CSPG 3PROSITE-ProRule annotationAdd BLAST112
Repeati689 – 796CSPG 4PROSITE-ProRule annotationAdd BLAST108
Repeati817 – 908CSPG 5PROSITE-ProRule annotationAdd BLAST92
Repeati934 – 1026CSPG 6PROSITE-ProRule annotationAdd BLAST93
Repeati1055 – 1157CSPG 7PROSITE-ProRule annotationAdd BLAST103
Repeati1178 – 1271CSPG 8PROSITE-ProRule annotationAdd BLAST94
Repeati1292 – 1388CSPG 9PROSITE-ProRule annotationAdd BLAST97
Repeati1409 – 1501CSPG 10PROSITE-ProRule annotationAdd BLAST93
Repeati1521 – 1610CSPG 11PROSITE-ProRule annotationAdd BLAST90
Repeati1644 – 1741CSPG 12PROSITE-ProRule annotationAdd BLAST98
Domaini1748 – 1847Calx-beta 1Add BLAST100
Domaini1860 – 1971Calx-beta 2Add BLAST112
Domaini1986 – 2092Calx-beta 3Add BLAST107
Domaini2107 – 2209Calx-beta 4Add BLAST103
Domaini2227 – 2331Calx-beta 5Add BLAST105

Domaini

The Calx-beta domains bind calcium with high affinity and undergo a major conformational shift upon binding.By similarity

Sequence similaritiesi

Belongs to the FRAS1 family.Curated

Keywords - Domaini

Repeat, Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1306 Eukaryota
KOG2090 Eukaryota
KOG3597 Eukaryota
ENOG410YBR4 LUCA
GeneTreeiENSGT00550000074429
HOGENOMiHOG000007464
HOVERGENiHBG081537
InParanoidiQ6NVD0
OMAiGHIMNQL
OrthoDBiEOG091G02YT
PhylomeDBiQ6NVD0
TreeFamiTF316876

Family and domain databases

Gene3Di2.60.40.2030, 5 hits
InterProiView protein in InterPro
IPR038081 CalX-like_sf
IPR003644 Calx_beta
PfamiView protein in Pfam
PF03160 Calx-beta, 5 hits
SMARTiView protein in SMART
SM00237 Calx_beta, 5 hits
SUPFAMiSSF141072 SSF141072, 5 hits
PROSITEiView protein in PROSITE
PS51854 CSPG, 12 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

Isoform 1 (identifier: Q6NVD0-1) [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
MASRARRTAK FSSFQPILAQ SPRLLLLLLL LSLVSYVSTQ AAGPGAALQS
60 70 80 90 100
LGLSGTSGVP TEEAIVVANR GLRVPFGREV WLDPLRDLVL QVQPGDRCTV
110 120 130 140 150
TVLDNDALAQ RPGHLSPKRF ACDYGPGEVR YSHLGARSPS RDRVRLQLRY
160 170 180 190 200
DAPGGAIVLP LALEVEVVFT QLEIVTRNLP LVVEELLGTS NALDDRSLEF
210 220 230 240 250
AYQPETEECR VGILSGLSAL PRYGELLHYP QVQGGAGDRG TSKTLLMDCK
260 270 280 290 300
AFQELGVRYR HTAPSRSPNR DWLPMVVELH SRGAPEGSPA LKREHFQVLV
310 320 330 340 350
RIRGGAENTA PKPSFVAMMM MEVDQFVLTA LTPDMLAAED AESDPDLLIF
360 370 380 390 400
NLTSAFQPGQ GYLVSTDDRS LPLSSFTQRD LRLLKIAYQP PSEDSDQERL
410 420 430 440 450
FELELEIVDP EGAASDPFAF MVVVKPMNTL APVVTRNTGL ILYEGQYRPL
460 470 480 490 500
TGPIGSGPQN LVISDEDDLE AVRLEVVAGL RHGHLVILGS PSSDSAPKTF
510 520 530 540 550
TVAELAAGQV VYQHDDKDGS LSDNLVLRMS DGGGRHQVQF LFPITLVPVD
560 570 580 590 600
DQPPVLNANT GLTVAEGETV PIPPLTLSAT DIDSDDSQLV FVLLPPFSSL
610 620 630 640 650
GHLLLRQRHV PQEEQGLWQK QGSFYERTVT EWRQQDITEG KLFYRHSGPH
660 670 680 690 700
SPGPVMDQFM FRVQDNHDPP NQSGIQRFVI RIHPVDRLPP ELGSGCPLRM
710 720 730 740 750
VVQESQLTPL RKRWLHYTDL DTDDRELQYT VTQPPTDTDE NHSPAPLGTL
760 770 780 790 800
VFTDNPSVVV SHFTQAQVNH HKIAYRPPGQ ELGVAARVAQ FQFQVEDRAG
810 820 830 840 850
NVAPGTFTLY LQPVDNQPPE IVNTGFTVEE KGHHILRETE LHVSDVDTDV
860 870 880 890 900
THISFTLTQA PKHGHMQISG RPLHVGGQFH LEDIKHGRIS YWNSGDESLT
910 920 930 940 950
DSCSLEVSDR HHVVPITLRV NVRPGDREGP MSVLPAGTLE SYLDVLENGA
960 970 980 990 1000
TEVTANIIKG AYQGTDDLML TFLLEGPPSY GEILVNGAPA EQFTQRDILE
1010 1020 1030 1040 1050
GSVVYAHTSG EIGLLPKADS FNLSLSAMSQ EWRIGSSIVQ GVTVWVTILP
1060 1070 1080 1090 1100
VDSQAPEISL GEQFVVLEGD KSVISLTHLS AEDMDSLKDD LLCTIVIQPT
1110 1120 1130 1140 1150
SGYVENISPA PGSEKSRAGV AISAFTLKDL RQGHINYVQS VHRGVEPVED
1160 1170 1180 1190 1200
RFIFRCSDGI NFSERQIFPI VIIPTNDEQP EMFMREFMVM EGMSLVVNRL
1210 1220 1230 1240 1250
ILNAADADIP RDDLTFTITR FPTHGHVMNQ LINGTVLVES FTLDQIIESS
1260 1270 1280 1290 1300
SIIYEHDDSE TQEDSFVIKL TDGKHSVEKM VLIVVIPVDD ETPRMTINNG
1310 1320 1330 1340 1350
LEIEIGETKV INNKVLMATD LDSDDKSLVY IIRYGPGHGL LQRQKPLGAF
1360 1370 1380 1390 1400
ENITLGMNFT QDEVDRNLIQ YVHFGQEGIR DLIKFDVTDG TNALIDRYFY
1410 1420 1430 1440 1450
VTIGSVDIVF PDVVSKGVSL KEGGKVTLTT DLLSTSDLNS PDENLVFTIT
1460 1470 1480 1490 1500
RAPMRGHLEC TDRRGLSITS FTQLQLAGNK IYYIHTAEDE VKMDSFEFQV
1510 1520 1530 1540 1550
TDGRNPVFRT FRISISDVDN KKPVVTIHNL VVSESESKLI TPFELTVEDR
1560 1570 1580 1590 1600
DTPDRLLKFI VTQVPVHGHL LFNNTRSVMV FTKQDLNENL ISYKHDGTES
1610 1620 1630 1640 1650
TEDSFSFTVT DGTHSDFYVF PDTVFETRRP QVMKIQVLPV DNSVPQIVVN
1660 1670 1680 1690 1700
KGASTLRTLA TGHLGFMITS KILKVEDRDS LHFSLRFIVT EAPQHGYLLN
1710 1720 1730 1740 1750
LGQGNHSVTQ FTQADIDDMK ICYVLRERAN ATSDMFHFIV EDDGGNRLTN
1760 1770 1780 1790 1800
QHFRLNWAWI SFEKEYYLIN EDSKFLDIVL TRRGYLGETS FISIGTRDGT
1810 1820 1830 1840 1850
AEKDRDFKGK AQKQVQFNPG QTRASWRVRI LSDGEHEHSE TFQVVLSEPV
1860 1870 1880 1890 1900
LAILEFPTVT TVEIIDPGDE STVFIPQSEY SVEEDVGELF IPIRRSGDIS
1910 1920 1930 1940 1950
RELMVICYTQ QGTATSTVRT SVLSYSDYIS RPEDHSSVIR FDKDEREKMC
1960 1970 1980 1990 2000
RILVIDDSLY EEEETFQVLL SMPMGGRIGD KFPGANVTIL TDRDDEPAFY
2010 2020 2030 2040 2050
FGDTQYSVDE SAGYVELQVW RTGTDLSKPS SVTVRSRKTE SLSADAGTDY
2060 2070 2080 2090 2100
VGISRNLDFA PGVNMQTVRV VILDDLGRPI LEGIEKFELV LRMPMNAALG
2110 2120 2130 2140 2150
EPSKATVSIN DSASDLPKMQ FKERVYTCNE NDGRVVAMIY RSGDIQHRSS
2160 2170 2180 2190 2200
VRCYTRQGSA QVMMDFEERP NTDVSTVTFL PGEMEKPCVL ELMDDAVYED
2210 2220 2230 2240 2250
VEELRLVLGT PQGSSAFGAA VGEQNETLIK IQDEADKAVI KFGETKFSVT
2260 2270 2280 2290 2300
EPSRPGESVV VKIPVIRQGD TSKVSIVRVH TKDGSATSGE DYHPVSEEIE
2310 2320 2330 2340 2350
FKEGETQHTV EIEVIFDGVR EMREAFTVHL KPDENMVAET QATKAIVYIE
2360 2370 2380 2390 2400
EIHSMADVTF PSVPHIVSLL IYDDPSKGRE DTGPVSGYPV VCITACNPKY
2410 2420 2430 2440 2450
PDYEKTGSIC ASENINDTLT RYRWLISAPA GPDGVTSPMR EVDFDTFFTS
2460 2470 2480 2490 2500
SKMITLDSIY FQPGSRVQCA ARAVNTNGNE GLELMSPIVT IGREEGLCQP
2510 2520 2530 2540 2550
RVPGVVGAEP FSAKLRYTGP EDPDFANLIK LTVTMPHIDG MLPAISTREL
2560 2570 2580 2590 2600
SNFELTLSPD GTRVGNHKCS NLLDYNEVKT HHGFLTNATK NPEVIGETYP
2610 2620 2630 2640 2650
YQYSVPVRGS STLRFYRNLN LEACLWEFVS YYDMTELLAD CGGTIGTDGQ
2660 2670 2680 2690 2700
VLNLVQSYVT LRVPLYVSYV FHSPVGVGGW QHFDLKSELR LTFVYDTAIL
2710 2720 2730 2740 2750
WNHGIGSPPE AELQGSLYPT SMRIGEEGRL AVNFKTEAQF HGLFVLSHPA
2760 2770 2780 2790 2800
SFTSSLIVSA DHPGLTFSLR LIRSEPTYNQ PVQQWSFVSD FAVRDYSGTY
2810 2820 2830 2840 2850
TVKLVPCTTP SNQEYRLPVT CNPREPVTFD LDIRFQQVSD PVATEFSLNT
2860 2870 2880 2890 2900
HMYLLSKKNL WLSDGSMGFG QESDVAFAEG DVIYGRVMVD PVQNLGDSFY
2910 2920 2930 2940 2950
CSIEKVFLCT GDDGYVPKYS PANAEYGCLA DSPSLLHRFK IVDKAQPETQ
2960 2970 2980 2990 3000
ATSFGDVLFN AKLAVDDPEA VLLVNQPGSD GFKVDSTPLF QVALGREWYI
3010 3020 3030 3040 3050
HTIYTVKSKD NTHRGIGKRS LEYQYHSVVH PGPPQATTKS WKKRAVRSTP
3060 3070 3080 3090 3100
SLAGEIGAEN NRGTNIQHIS LNRRGKRQVP HGRIPPDGIL PWELNSPSSE
3110 3120 3130 3140 3150
VSLVTVLGGL TVGLLTVCLA VAAAVMCRNR STKGKDTPKG SGSTEPMMSP
3160
QSHYNDSSEV
Length:3,160
Mass (Da):350,640
Last modified:August 16, 2005 - v2
Checksum:i579F20BD79957E06
GO
Isoform 2 (identifier: Q6NVD0-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-2287: Missing.
     2288-2297: SGEDYHPVSE → MVGPGNFRKR

Note: No experimental confirmation available.
Show »
Length:873
Mass (Da):96,504
Checksum:i198D3BE068E6C0DB
GO
Isoform 3 (identifier: Q6NVD0-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-2851: Missing.

Note: No experimental confirmation available.
Show »
Length:309
Mass (Da):33,633
Checksum:iB0E805BEB9A456F1
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti2437S → N in BAC27425 (PubMed:16141072).Curated1
Sequence conflicti2851H → N in BAC27425 (PubMed:16141072).Curated1
Sequence conflicti3031P → H in AAH46388 (PubMed:15489334).Curated1
Sequence conflicti3040S → G in AAH46388 (PubMed:15489334).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0150371 – 2851Missing in isoform 3. 1 PublicationAdd BLAST2851
Alternative sequenceiVSP_0150381 – 2287Missing in isoform 2. 1 PublicationAdd BLAST2287
Alternative sequenceiVSP_0150392288 – 2297SGEDYHPVSE → MVGPGNFRKR in isoform 2. 1 Publication10

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AJ833643 mRNA Translation: CAH55760.1
AK031494 mRNA Translation: BAC27425.1
AK082256 mRNA Translation: BAC38448.1
BC046388 mRNA Translation: AAH46388.1
BC068185 mRNA Translation: AAH68185.1
AB160988 mRNA Translation: BAD89016.1
CCDSiCCDS17348.1 [Q6NVD0-1]
RefSeqiNP_766450.2, NM_172862.3 [Q6NVD0-1]
UniGeneiMm.38378

Genome annotation databases

EnsembliENSMUST00000091137; ENSMUSP00000088670; ENSMUSG00000037016 [Q6NVD0-1]
GeneIDi242022
KEGGimmu:242022
UCSCiuc008pex.1 mouse [Q6NVD0-3]
uc008pey.1 mouse [Q6NVD0-1]

Keywords - Coding sequence diversityi

Alternative splicing

Similar proteinsi

Entry informationi

Entry nameiFREM2_MOUSE
AccessioniPrimary (citable) accession number: Q6NVD0
Secondary accession number(s): Q4W2Q5
, Q5H8C0, Q811G9, Q8C4G5, Q8CD46
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 16, 2005
Last sequence update: August 16, 2005
Last modified: March 28, 2018
This is version 116 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
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health