FREM2_HUMAN » FRAS1-related extracellular matrix protein 2

FREM2_HUMAN » FRAS1-related extracellular matrix protein 2
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
FREM2_HUMAN » FRAS1-related extracellular matrix protein 2 » ECM3 homolog;
Hydrophobic Thickness 38.0 ± 4.8 Å
Tilt Angle 22 ± 17°
ΔGtransfer -22.7 kcal/mol
ΔGfold -14.6 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, HMDB
Topology Out
TM Segments 3107-3135 (3105-3139)
Pathways none
PDB none
OPM none
Complexes none
Interactions

RYK, Complex: RYK:FREM2, PubMed

Domains

AA: 302-416, Cadherin-like

AA: 421-539, Cadherin-like

AA: 543-677, Cadherin-like

AA: 683-809, Cadherin-like

AA: 812-921, Cadherin-like

AA: 923-1039, Cadherin-like

AA: 1050-1170, Cadherin-like

AA: 1173-1284, Cadherin-like

AA: 1286-1401, Cadherin-like

AA: 1405-1514, Cadherin-like

AA: 1516-1623, Cadherin-like

AA: 1638-1754, Cadherin-like

AA: 1762-1858, PDBID: 2DPK, Subunit A, Seq Identity:29%, Calx-beta domain

AA: 1871-1982, PDBID: 2DPK, Subunit A, Seq Identity:32%, Calx-beta domain

AA: 1996-2103, PDBID: 3E9T, Subunit D, Seq Identity:34%, Calx-beta domain

AA: 2116-2220, PDBID: 3E9T, Subunit D, Seq Identity:28%, Calx-beta domain

AA: 2238-2342, PDBID: 3E9T, Subunit D, Seq Identity:31%, Calx-beta domain

UniProt annotation for FREM2_HUMAN » FRAS1-related extracellular matrix protein 2
FUNCTION: 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.

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

DISEASE: Fraser syndrome (FRASS) OMIM: Multisystem malformation usually comprising cryptophthalmos, cutaneous syndactyly, ear abnormalities, renal agenesis and congenital heart defects. by mutations affecting the gene represented in this entry.

UniProt features for FREM2_HUMAN » FRAS1-related extracellular matrix protein 2
SIGNAL 1 46 Potential.
CHAIN 47 3169 FRAS1-related extracellular matrix protein 2.
REPEAT 298 419 CSPG 1.
REPEAT 420 541 CSPG 2.
REPEAT 542 681 CSPG 3.
REPEAT 682 811 CSPG 4.
REPEAT 812 921 CSPG 5.
REPEAT 922 1052 CSPG 6.
REPEAT 1053 1171 CSPG 7.
REPEAT 1172 1284 CSPG 8.
REPEAT 1285 1400 CSPG 9.
REPEAT 1401 1514 CSPG 10.
REPEAT 1515 1633 CSPG 11.
REPEAT 1634 1758 CSPG 12.
DOMAIN 1759 1858 Calx-beta 1.
DOMAIN 1871 1982 Calx-beta 2.
DOMAIN 1997 2103 Calx-beta 3.
DOMAIN 2118 2220 Calx-beta 4.
DOMAIN 2238 2342 Calx-beta 5.
Amino Acid Sequence for FREM2_HUMAN » FRAS1-related extracellular matrix protein 2
MHSAGTPGLS SRRTGNSTSF QPGPPPPPRL LLLLLLLLSL VSRVPAQPAA FGRALLSPGL AGAAGVPAEE AIVLANRGLR VPFGREVWLD PLHDLVLQVQ PGDRCAVSVL DNDALAQRPG RLSPKRFPCD FGPGEVRYSH LGARSPSRDR VRLQLRYDAP GGAVVLPLVL EVEVVFTQLE VVTRNLPLVV EELLGTSNAL DARSLEFAFQ PETEECRVGI LSGLGALPRY GELLHYPQVP GGAREGGAPE TLLMDCKAFQ ELGVRYRHTA ASRSPNRDWI PMVVELRSRG APVGSPALKR EHFQVLVRIR GGAENTAPKP SFVAMMMMEV DQFVLTALTP DMLAAEDAES PSDLLIFNLT SPFQPGQGYL VSTDDRSLPL SSFTQRDLRL LKIAYQPPSE DSDQERLFEL ELEVVDLEGA ASDPFAFMVV VKPMNTMAPV VTRNTGLILY EGQSRPLTGP AGSGPQNLVI SDEDDLEAVR LEVVAGLRHG HLVILGASSG SSAPKSFTVA ELAAGQVVYQ HDDRDGSLSD NLVLRMVDGG GRHQVQFLFP ITLVPVDDQP PVLNANTGLT LAEGETVPIL PLSLSATDMD SDDSLLLFVL ESPFLTTGHL LLRQTHPPHE KQELLRGLWR KEGAFYERTV TEWQQQDITE GRLFYRHSGP HSPGPVTDQF TFRVQDNHDP PNQSGLQRFV IRIHPVDRLP PELGSGCPLR MVVQESQLTP LRKKWLRYTD LDTDDRELRY TVTQSPTDTD ENHLPAPLGT LVLTDNPSVV VTHFTQAQIN HHKIAYRPPG QELGVATRVA QFQFQVEDRA GNVAPGTFTL YLHPVDNQPP EILNTGFTIQ EKGHHILSET ELHVNDVDTD VAHISFTLTQ APKHGHMRVS GQILHVGGLF HLEDIKQGRV SYAHNGDKSL TDSCSLEVSD RHHVVPITLR VNVRPVDDEV PILSHPTGTL ESYLDVLENG ATEITANVIK GTNEETDDLM LTFLLEDPPL YGEILVNGIP AEQFTQRDIL EGSVVYTHTS GEIGLLPKAD SFNLSLSDMS QEWRIGGNTI QGVTIWVTIL PVDSQAPEIF VGEQLIVMEG DKSVITSVHI SAEDVDSLND DILCTIVIQP TSGYVENISP APGSEKSRAG IAISAFNLKD LRQGHINYVQ SVHKGVEPVE DRFVFRCSDG INFSERQFFP IVIIPTNDEQ PEMFMREFMV MEGMSLVIDT PILNAADADV PLDDLTFTIT QFPTHGHIMN QLINGTVLVE SFTLDQIIES SSIIYEHDDS ETQEDSFVIK LTDGKHSVEK TVLIIVIPVD DETPRMTINN GLEIEIGDTK IINNKILMAT DLDSEDKSLV YIIRYGPGHG LLQRRKPTGA FENITLGMNF TQDEVDRNLI QYVHLGQEGI RDLIKFDVTD GINPLIDRYF YVSIGSIDIV FPDVISKGVS LKEGGKVTLT TDLLSTSDLN SPDENLVFTI TRAPMRGHLE CTDQPGVSIT SFTQLQLAGN KIYYIHTADD EVKMDSFEFQ VTDGRNPVFR TFRISISDVD NKKPVVTIHK LVVSESENKL ITPFELTVED RDTPDKLLKF TITQVPIHGH LLFNNTRPVM VFTKQDLNEN LISYKHDGTE SSEDSFSFTV TDGTHTDFYV FPDTVFETRR PQVMKIQVLA VDNSVPQIAV NKGASTLRTL ATGHLGFMIT SKILKVEDRD SLHISLRFIV TEAPQHGYLL NLDKGNHSIT QFTQADIDDM KICYVLREGA NATSDMFYFA VEDGGGNKLT YQNFRLNWAW ISFEKEYYLV NEDSKFLDVV LKRRGYLGET SFISIGTRDR TAEKDKDFKG KAQKQVQFNP GQTRATWRVR ILSDGEHEQS ETFQVVLSEP VLAALEFPTV ATVEIVDPGD EPTVFIPQSK YSVEEDVGEL FIPIRRSGDV SQELMVVCYT QQGTATGTVP TSVLSYSDYI SRPEDHTSVV RFDKDEREKL CRIVIIDDSL YEEEETFHVL LSMPMGGRIG SEFPGAQVTI VPDKDDEPIF YFGDVEYSVD ESAGYVEVQV WRTGTDLSKS SSVTVRSRKT DPPSADAGTD YVGISRNLDF APGVNMQPVR VVILDDLGQP ALEGIEKFEL VLRMPMNAAL GEPSKATVSI NDSVSDLPKM QFKERIYTGS ESDGQIVTMI HRTGDVQYRS SVRCYTRQGS AQVMMDFEER PNTDTSIITF LPGETEKPCI LELMDDVLYE EVEELRLVLG TPQSNSPFGA AVGEQNETLI RIRDDADKTV IKFGETKFSV TEPKEPGESV VIRIPVIRQG DTSKVSIVRV HTKDGSATSG EDYHPVSEEI EFKEGETQHV VEIEVTFDGV REMREAFTVH LKPDENMIAE MQLTKAIVYI EEMSSMADVT FPSVPQIVSL LMYDDTSKAK ESAEPMSGYP VICITACNPK YSDYDKTGSI CASENINDTL TRYRWLISAP AGPDGVTSPM REVDFDTFFT SSKMVTLDSI YFQPGSRVQC AARAVNTNGD EGLELMSPIV TISREEGLCQ PRVPGVVGAE PFSAKLRYTG PEDADYTNLI KLTVTMPHID GMLPVISTRE LSNFELTLSP DGTRVGNHKC SNLLDYTEVK THYGFLTDAT KNPEIIGETY PYQYSLSIRG STTLRFYRNL NLEACLWEFV SYYDMSELLA DCGGTIGTDG QVLNLVQSYV TLRVPLYVSY VFHSPVGVGG WQHFDLKSEL RLTFVYDTAI LWNDGIGSPP EAELQGSLYP TSMRIGDEGR LAVHFKTEAQ FHGLFVLSHP ASFTSSVIMS ADHPGLTFSL RLIRSEPTYN QPVQQWSFVS DFAVRDYSGT YTVKLVPCTA PSHQEYRLPV TCNPREPVTF DLDIRFQQVS DPVAAEFSLN TQMYLLSKKS LWLSDGSMGF GQESDVAFAE GDIIYGRVMV DPVQNLGDSF YCSIEKVFLC TGADGYVPKY SPMNAEYGCL ADSPSLLYRF KIVDKAQPET QATSFGNVLF NAKLAVDDPE AILLVNQPGS DGFKVDSTPL FQVALGREWY IHTIYTVRSK DNANRGIGKR SVEYHSLVSQ GKPQSTTKSR KKREIRSTPS LAWEIGAENS RGTNIQHIAL DRTKRQIPHG RAPPDGILPW ELNSPSSAVS LVTVVGGTTV GLLTICLTVI AVLMCRGKES FRGKDAPKGS SSSEPMVPPQ SHHNDSSEV