FRAS1_HUMAN » Extracellular matrix protein FRAS1

FRAS1_HUMAN » Extracellular matrix protein FRAS1
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Topology in Plasma membrane
Topologyextracellular side
cytoplasmic side
FRAS1_HUMAN » Extracellular matrix protein FRAS1 »
Hydrophobic Thickness 34.4 ± 2.8 Å
Tilt Angle 0 ± 0°
ΔGtransfer -26.8 kcal/mol
ΔGfold -23.0 kcal/mol
Links UniProtKB, Pfam, Interpro, iHOP, STRING, HGNC, HMDB
Topology Out
TM Segments 3898-3921 (3896-3924)
Pathways none
PDB none
OPM none
Complexes none
Interactions none
Domains

AA: 95-152, PDBID: 1U5M, Subunit A, Seq Identity:31%, von Willebrand factor type C domain

AA: 159-216, PDBID: 1U5M, Subunit A, Seq Identity:28%, von Willebrand factor type C domain

AA: 221-278, PDBID: 1U5M, Subunit A, Seq Identity:31%, von Willebrand factor type C domain

AA: 285-342, PDBID: 1U5M, Subunit A, Seq Identity:33%, von Willebrand factor type C domain

AA: 1099-1199, Cadherin-like

AA: 1203-1310, Cadherin-like

AA: 1313-1440, Cadherin-like

AA: 1446-1574, Cadherin-like

AA: 1577-1691, Cadherin-like

AA: 1694-1812, Cadherin-like

AA: 1816-1938, Cadherin-like

AA: 1941-2059, Cadherin-like

AA: 2067-2179, Cadherin-like

AA: 2184-2293, Cadherin-like

AA: 2295-2406, Cadherin-like

AA: 2422-2538, Cadherin-like

AA: 2547-2646, PDBID: 3E9T, Subunit D, Seq Identity:34%, Calx-beta domain

AA: 2659-2770, PDBID: 2DPK, Subunit A, Seq Identity:26%, Calx-beta domain

AA: 2784-2890, PDBID: 3E9T, Subunit D, Seq Identity:31%, Calx-beta domain

AA: 2903-3007, PDBID: 3E9T, Subunit D, Seq Identity:28%, Calx-beta domain

AA: 3025-3129, PDBID: 3E9T, Subunit D, Seq Identity:31%, Calx-beta domain

UniProt annotation for FRAS1_HUMAN » Extracellular matrix protein FRAS1
TISSUE SPECIFICITY: Expressed in many adult tissues, with highest levels in kidney, pancreas and thalamus. Relatively high expression was also detected in fetal kidney and heart.

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 FRAS1_HUMAN » Extracellular matrix protein FRAS1
SIGNAL 1 26 Potential.
CHAIN 27 4008 Extracellular matrix protein FRAS1.
DOMAIN 27 88 VWFC 1.
DOMAIN 93 153 VWFC 2.
DOMAIN 157 217 VWFC 3.
DOMAIN 219 279 VWFC 4.
DOMAIN 283 343 VWFC 5.
DOMAIN 347 417 VWFC 6.
REPEAT 409 460 FU 1.
REPEAT 462 505 FU 2.
REPEAT 507 553 FU 3.
REPEAT 555 599 FU 4.
REPEAT 602 647 FU 5.
REPEAT 649 705 FU 6.
REPEAT 708 753 FU 7.
REPEAT 755 800 FU 8.
REPEAT 803 852 FU 9.
REPEAT 854 900 FU 10.
REPEAT 903 948 FU 11.
REPEAT 952 997 FU 12.
REPEAT 999 1042 FU 13.
REPEAT 1046 1089 FU 14.
REPEAT 1090 1201 CSPG 1.
REPEAT 1202 1310 CSPG 2.
REPEAT 1311 1445 CSPG 3.
REPEAT 1446 1574 CSPG 4.
REPEAT 1575 1691 CSPG 5.
REPEAT 1692 1821 CSPG 6.
REPEAT 1822 1939 CSPG 7.
REPEAT 1940 2059 CSPG 8.
REPEAT 2060 2178 CSPG 9.
REPEAT 2179 2293 CSPG 10.
REPEAT 2294 2415 CSPG 11.
REPEAT 2418 2542 CSPG 12.
DOMAIN 2543 2646 Calx-beta 1.
DOMAIN 2659 2770 Calx-beta 2.
DOMAIN 2784 2890 Calx-beta 3.
DOMAIN 2905 3007 Calx-beta 4.
DOMAIN 3025 3129 Calx-beta 5.
Amino Acid Sequence for FRAS1_HUMAN » Extracellular matrix protein FRAS1
MGVSKVWLGL ALALAEFAVL PHHSEGACVY QGSLLADATI WKPDSCQSCR CHGDIVICKP AVCRNPQCAF EKGEVLQIAA NQCCPECVLR TPGSCHHEKK IHEHGTEWAS SPCSVCSCNH GEVRCTPQPC PPLSCGHQEL AFIPEGSCCP VCVGLGKPCS YEGHVFQDGE DWRLSRCAKC LCRNGVAQCF TAQCQPLFCN QDETVVRVPG KCCPQCSARS CSAAGQVYEH GEQWSENACT TCICDRGEVR CHKQACLPLR CGKGQSRARR HGQCCEECVS PAGSCSYDGV VRYQDEMWKG SACEFCMCDH GQVTCQTGEC AKVECARDEE LIHLDGKCCP ECISRNGYCV YEETGEFMSS NASEVKRIPE GEKWEDGPCK VCECRGAQVT CYEPSCPPCP VGTLALEVKG QCCPDCTSVH CHPDCLTCSQ SPDHCDLCQD PTKLLQNGWC VHSCGLGFYQ AGSLCLACQP QCSTCTSGLE CSSCQPPLLM RHGQCVPTCG DGFYQDRHSC AVCHESCAGC WGPTEKHCLA CRDPLHVLRD GGCESSCGKG FYNRQGTCSA CDQSCDSCGP SSPRCLTCTE KTVLHDGKCM SECPGGYYAD ATGRCKVCHN SCASCSGPTP SHCTACSPPK ALRQGHCLPR CGEGFYSDHG VCKACHSSCL ACMGPAPSHC TGCKKPEEGL QVEQLSGVGI PSGECLAQCR AHFYLESTGL CEACHQSCFR CAGKSPHNCT DCGPSHVLLD GQCLSQCPDG YFHQEGSCTE CHPTCRQCHG PLESDCISCY PHISLTNGNC RTSCREEQFL NLVGYCADCH HLCQHCAADL HNTGSICLRC QNAHYLLLGD HCVPDCPSGY YAERGACKKC HSSCRTCQGR GPFSCSSCDT NLVLSHTGTC STTCFPGHYL DDNHVCQPCN THCGSCDSQA SCTSCRDPNK VLLFGECQYE SCAPQYYLDF STNTCKECDW SCSACSGPLK TDCLQCMDGY VLQDGACVEQ CLSSFYQDSG LCKCDSYCLQ CQGPHECTRC KGPFLLLEAQ CVQECGKGYF ADHAKHKCTA CPQGCLQCSH RDRCHLCDHG FFLKSGLCVY NCVPGFSVHT SNETCSGKIH TPSLHVNGSL ILPIGSIKPL DFSLLNVQDQ EGRVEDLLFH VVSTPTNGQL VLSRNGKEVQ LDKAGRFSWK DVNEKKVRFV HSKEKLRKGY LFLKISDQQF FSEPQLINIQ AFSTQAPYVL RNEVLHISRG ERATITTQML DIRDDDNPQD VVIEIIDPPL HGQLLQTLQS PATPIYQFQL DELSRGLLHY AHDGSDSTSD VAVLQANDGH SFHNILFQVK TVPQNDRGLQ LVANSMVWVP EGGMLQITNR ILQAEAPGAS AEEIIYKITQ DYPQFGEVVL LVNMPADSPA DEGQHLPDGR TATPTSTFTQ QDINEGIVWY RHSGAPAQSD SFRFEVSSAS NAQTRLESHM FNIAILPQTP EAPKVSLEAS LHMTAREDGL TVIQPHSLSF INSEKPSGKI VYNITLPLHP NQGIIEHRDH PHSPIRYFTQ EDINQGKVMY RPPPAAPHLQ ELMAFSFAGL PESVKFHFTV SDGEHTSPEM VLTIHLLPSD QQLPVFQVTA PRLAVSPGGS TSVGLQVVVR DAETAPKELF FELRRPPQHG VLLKHTAEFR RPMATGDTFT YEDVEKNALQ YIHDGSSTRE DSMEISVTDG LTVTMLEVRV EVSLSEDRGP RLAAGSSLSI TVASKSTAII TRSHLAYVDD SSPDPEIWIQ LNYLPSYGTL LRISGSEVEE LSEVSNFTME DINNKKIRYS AVFETDGHLV TDSFYFSVSD MDHNHLDNQI FTIMITPAEN PPPVIAFADL ITVDEGGRAP LSFHHFFATD DDDNLQRDAI IKLSALPKYG CIENTGTGDR FGPETASDLE ASFPIQDVLE NYIYYFQSVH ESIEPTHDIF SFYVSDGTSR SEIHSINITI ERKNDEPPRM TLQPLRVQLS SGVVISNSSL SLQDLDTPDN ELIFVLTKKP DHGHVLWRQT ASEPLENGRV LVQGSTFTYQ DILAGLVGYV PSVPGMVVDE FQFSLTDGLH VDTGRMKIYT ELPASDTPHL AINQGLQLSA GSVARITEQH LKVTDIDSDD HQVMYIMKED PGAGRLQMMK HGNLEQISIK GPIRSFTQAD ISQGQPEYSH GTGEPGGSFA FKFDVVDGEG NRLIDKSFSI SISEDKSPPV ITTNKGLVLD ENSVKKITTL QLSATDQDSG PTELIYRITR QPQLGHLEHA ASPGIQISSF TQADLTSRNV QYVHSSEAEK HSDAFSFTLS DGVSEVTQTF HITLHPVDDS LPVVQNLGMR VQEGMRKTIT EFELKAVDAD TEAESVTFTI VQPPRHGTIE RTSNGQHFHL TSTFTMKDIY QNRVSYSHDG SNSLKDRFTF TVSDGTNPFF IIEEGGKEIM TAAPQPFRVD ILPVDDGTPR IVTNLGLQWL EYMDGKATNL ITKKELLTMD PDTEDAQLVY EITTGPKHGF VENKLQPGRA AATFTQEDVN LGLIRYVLHK EKIREMMDSF QFLVKDSKPN VVSDNVFHIQ WSLISFKYTS YNVSEKAGSV SVTVQRTGNL NQYAIVLCRT EQGTASSSSQ PGQQDYVEYA GQVQFDERED TKSCTIVIND DDVFENVESF TVELSMPAYA LLGEFTQAKV IINDTEDEPT LEFDKKIYWV NESAGFLFAP IERKGDASSI VSAICYTVPK SAMGSSFYAL ESGSDFKSRG MSAASRVIFG PGVTMSTCDV MLIDDSEYEE EEEFEIALAD ASDNARIGRV ATAKVLISGP NDASTVSLGN TAFTVSEDAG TVKIPVIRHG TDLSTFASVW CATRPSDPAS ATPGVDYVPS SRKVEFGPGV IEQYCTLTIL DDTQYPVIEG LETFVVFLSS AQGAELTKPF QAVIAINDTF QDVPSMQFAK DLLLVKEKEG VLHVPITRSG DLSYESSVRC YTQSHSAQVM EDFEERQNAD SSRITFLKGD KVKNCTVYIH DDSMFEPEEQ FRVYLGLPLG NHWSGARIGK NNMATITISN DEDAPTIEFE EAAYQVREPA GPDAIAILNI KVIRRGDQNR TSKVRCSTRD GSAQSGVDYY PKSRVLKFSP GVDHIFFKVE ILSNEDREWH ESFSLVLGPD DPVEAVLGDV TTATVTILDQ EAAGSLILPA PPIVVTLADY DHVEEVTKEG VKKSPSPGYP LVCVTPCDPH FPRYAVMKER CSEAGINQTS VQFSWEVAAP TDGNGARSPF ETITDNTPFT SVNHMVLDSI YFSRRFHVRC VAKAVDKVGH VGTPLRSNIV TIGTDSAICH TPVVAGTSRG FQAQSFIATL KYLDVKHKEH PNRIHISVQI PHQDGMLPLI STMPLHNLHF LLSESIYRHQ HVCSNLVTTY DLRGLAEAGF LDDVVYDSTA LGPGYDRPFQ FDPSVREPKT IQLYKHLNLK SCVWTFDAYY DMTELIDVCG GSVTADFQVR DSAQSFLTVH VPLYVSYIYV TAPRGWASLE HHTEMEFSFF YDTVLWRTGI QTDSVLSARL QIIRIYIRED GRLVIEFKTH AKFRGQFVME HHTLPEVKSF VLTPDHLGGI EFDLQLLWSA QTFDSPHQLW RATSSYNRKD YSGEYTIYLI PCTVQPTQPW VDPGEKPLAC TAHAPERFLI PIAFQQTNRP VPVVYSLNTE FQLCNNEKVF LMDPNTSDMS LAEMDYKGAF SKGQILYGRV LWNPEQNLNS AYKLQLEKVY LCTGKDGYVP FFDPTGTIYN EGPQYGCIQP NKHLKHRFLL LDRNQPEVTD KYFHDVPFEA HFASELPDFH VVSNMPGVDG FTLKVDALYK VEAGHQWYLQ VIYIIGPDTI SGPRVQRSLT APLRRNRRDL VEPDGQLILD DSLIYDNEGD QVKNGTNMKS LNLEMQELAV AASLSQTGAS IGSALAAIML LLLVFLVACF INRKCQKQRK KKPAEDILEE YPLNTKVEVP KRHPDRVEKN VNRHYCTVRN VNILSEPEAA YTFKGAKVKR LNLEVRVHNN LQDGTEV