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Index > Protein center > FBN2(Gene name) > Human
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  • FBN2 (Gene name),
  • Fibrillin-2 (Protein name ),  FBN2_HUMAN from NCBI database.
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  • General Annotation
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  • Antigen Annotation
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  • Gene name:
    FBN2;
    Protein name:
    Fibrillin-2;
    Alternative:

    Organism:
    Human (Homo sapiens). 
    General Annotation
    Sub Unit:
    N/A
    Function:
    Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively.
    Subcellular Location:
    Secreted extracellular space extracellular matrix
    Protein Attributes:
    Sequence length:
    2912
    Sequence:
    50:
    MGRRRRLCLQ | LYFLWLGCVV | LWAQGTAGQP | QPPPPKPPRP | QPPPQQVRSA | 
    100:
    TAGSEGGFLA | PEYREEGAAV | ASRVRRRGQQ | DVLRGPNVCG | SRFHSYCCPG | 
    150:
    WKTLPGGNQC | IVPICRNSCG | DGFCSRPNMC | TCSSGQISST | CGSKSIQQCS | 
    200:
    VRCMNGGTCA | DDHCQCQKGY | IGTYCGQPVC | ENGCQNGGRC | IGPNRCACVY | 
    250:
    GFTGPQCERD | YRTGPCFTQV | NNQMCQGQLT | GIVCTKTLCC | ATIGRAWGHP | 
    300:
    CEMCPAQPQP | CRRGFIPNIR | TGACQDVDEC | QAIPGICQGG | NCINTVGSFE | 
    350:
    CRCPAGHKQS | ETTQKCEDID | ECSIIPGICE | TGECSNTVGS | YFCVCPRGYV | 
    400:
    TSTDGSRCID | QRTGMCFSGL | VNGRCAQELP | GRMTKMQCCC | EPGRCWGIGT | 
    450:
    IPEACPVRGS | EEYRRLCMDG | LPMGGIPGSA | GSRPGGTGGN | GFAPSGNGNG | 
    500:
    YGPGGTGFIP | IPGGNGFSPG | VGGAGVGAGG | QGPIITGLTI | LNQTIDICKH | 
    550:
    HANLCLNGRC | IPTVSSYRCE | CNMGYKQDAN | GDCIDVDECT | SNPCTNGDCV | 
    600:
    NTPGSYYCKC | HAGFQRTPTK | QACIDIDECI | QNGVLCKNGR | CVNTDGSFQC | 
    650:
    ICNAGFELTT | DGKNCVDHDE | CTTTNMCLNG | MCINEDGSFK | CICKPGFVLA | 
    700:
    PNGRYCTDVD | ECQTPGICMN | GHCINSEGSF | RCDCPPGLAV | GMDGRVCVDT | 
    750:
    HMRSTCYGGI | KKGVCVRPFP | GAVTKSECCC | ANPDYGFGEP | CQPCPAKNSA | 
    800:
    EFHGLCSSGV | GITVDGRDIN | ECALDPDICA | NGICENLRGS | YRCNCNSGYE | 
    850:
    PDASGRNCID | IDECLVNRLL | CDNGLCRNTP | GSYSCTCPPG | YVFRTETETC | 
    900:
    EDINECESNP | CVNGACRNNL | GSFNCECSPG | SKLSSTGLIC | IDSLKGTCWL | 
    950:
    NIQDSRCEVN | INGATLKSEC | CATLGAAWGS | PCERCELDTA | CPRGLARIKG | 
    1000:
    VTCEDVNECE | VFPGVCPNGR | CVNSKGSFHC | ECPEGLTLDG | TGRVCLDIRM | 
    1050:
    EQCYLKWDED | ECIHPVPGKF | RMDACCCAVG | AAWGTECEEC | PKPGTKEYET | 
    1100:
    LCPRGAGFAN | RGDVLTGRPF | YKDINECKAF | PGMCTYGKCR | NTIGSFKCRC | 
    1150:
    NSGFALDMEE | RNCTDIDECR | ISPDLCGSGI | CVNTPGSFEC | ECFEGYESGF | 
    1200:
    MMMKNCMDID | ECERNPLLCR | GGTCVNTEGS | FQCDCPLGHE | LSPSREDCVD | 
    1250:
    INECSLSDNL | CRNGKCVNMI | GTYQCSCNPG | YQATPDRQGC | TDIDECMIMN | 
    1300:
    GGCDTQCTNS | EGSYECSCSE | GYALMPDGRS | CADIDECENN | PDICDGGQCT | 
    1350:
    NIPGEYRCLC | YDGFMASMDM | KTCIDVNECD | LNSNICMFGE | CENTKGSFIC | 
    1400:
    HCQLGYSVKK | GTTGCTDVDE | CEIGAHNCDM | HASCLNIPGS | FKCSCREGWI | 
    1450:
    GNGIKCIDLD | ECSNGTHQCS | INAQCVNTPG | SYRCACSEGF | TGDGFTCSDV | 
    1500:
    DECAENINLC | ENGQCLNVPG | AYRCECEMGF | TPASDSRSCQ | DIDECSFQNI | 
    1550:
    CVFGTCNNLP | GMFHCICDDG | YELDRTGGNC | TDIDECADPI | NCVNGLCVNT | 
    1600:
    PGRYECNCPP | DFQLNPTGVG | CVDNRVGNCY | LKFGPRGDGS | LSCNTEIGVG | 
    1650:
    VSRSSCCCSL | GKAWGNPCET | CPPVNSTEYY | TLCPGGEGFR | PNPITIILED | 
    1700:
    IDECQELPGL | CQGGNCINTF | GSFQCECPQG | YYLSEDTRIC | EDIDECFAHP | 
    1750:
    GVCGPGTCYN | TLGNYTCICP | PEYMQVNGGH | NCMDMRKSFC | YRSYNGTTCE | 
    1800:
    NELPFNVTKR | MCCCTYNVGK | AWNKPCEPCP | TPGTADFKTI | CGNIPGFTFD | 
    1850:
    IHTGKAVDID | ECKEIPGICA | NGVCINQIGS | FRCECPTGFS | YNDLLLVCED | 
    1900:
    IDECSNGDNL | CQRNADCINS | PGSYRCECAA | GFKLSPNGAC | VDRNECLEIP | 
    1950:
    NVCSHGLCVD | LQGSYQCICH | NGFKASQDQT | MCMDVDECER | HPCGNGTCKN | 
    2000:
    TVGSYNCLCY | PGFELTHNND | CLDIDECSSF | FGQVCRNGRC | FNEIGSFKCL | 
    2050:
    CNEGYELTPD | GKNCIDTNEC | VALPGSCSPG | TCQNLEGSFR | CICPPGYEVK | 
    2100:
    SENCIDINEC | DEDPNICLFG | SCTNTPGGFQ | CLCPPGFVLS | DNGRRCFDTR | 
    2150:
    QSFCFTNFEN | GKCSVPKAFN | TTKAKCCCSK | MPGEGWGDPC | ELCPKDDEVA | 
    2200:
    FQDLCPYGHG | TVPSLHDTRE | DVNECLESPG | ICSNGQCINT | DGSFRCECPM | 
    2250:
    GYNLDYTGVR | CVDTDECSIG | NPCGNGTCTN | VIGSFECNCN | EGFEPGPMMN | 
    2300:
    CEDINECAQN | PLLCAFRCMN | TFGSYECTCP | IGYALREDQK | MCKDLDECAE | 
    2350:
    GLHDCESRGM | MCKNLIGTFM | CICPPGMARR | PDGEGCVDEN | ECRTKPGICE | 
    2400:
    NGRCVNIIGS | YRCECNEGFQ | SSSSGTECLD | NRQGLCFAEV | LQTICQMASS | 
    2450:
    SRNLVTKSEC | CCDGGRGWGH | QCELCPLPGT | AQYKKICPHG | PGYTTDGRDI | 
    2500:
    DECKVMPNLC | TNGQCINTMG | SFRCFCKVGY | TTDISGTSCI | DLDECSQSPK | 
    2550:
    PCNYICKNTE | GSYQCSCPRG | YVLQEDGKTC | KDLDECQTKQ | HNCQFLCVNT | 
    2600:
    LGGFTCKCPP | GFTQHHTACI | DNNECGSQPS | LCGAKGICQN | TPGSFSCECQ | 
    2650:
    RGFSLDATGL | NCEDVDECDG | NHRCQHGCQN | ILGGYRCGCP | QGYIQHYQWN | 
    2700:
    QCVDENECSN | PNACGSASCY | NTLGSYKCAC | PSGFSFDQFS | SACHDVNECS | 
    2750:
    SSKNPCNYGC | SNTEGGYLCG | CPPGYYRVGQ | GHCVSGMGFN | KGQYLSLDTE | 
    2800:
    VDEENALSPE | ACYECKINGY | SKKDSRQKRS | IHEPDPTAVE | QISLESVDMD | 
    2850:
    SPVNMKFNLS | HLGSKEHILE | LRPAIQPLNN | HIRYVISQGN | DDSVFRIHQR | 
    2900:
    NGLSYLHTAK | KKLMPGTYTL | EITSIPLYKK | KELKKLEESN | EDDYLLGELG | 
    2912:
    EALRMRLQIQ | LY
    3D Structure:
    N/A
    Predicted Eptitope:
    Please Sign in.
    EIAab Sequence  Vaild Sequence:
    Please Sign in.
    Related Databases
    SMR:
    MIM:
    Pfam:
    String:
    Pfam:
    KEGG:
    UniGene:
    Uniprot:
     
    FOR
    ELISA Kit for Human Fibrillin-2
    Cat.:
    E8805h
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    Range:
    Please sign in first.
    ELISA Kit for Human Fibrillin-2
    Cat.:
    E8805m
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    CLIA Kit for Human Fibrillin-2
    Cat.:
    U8805h
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    CLIA Kit for Human Fibrillin-2
    Cat.:
    U8805m
    Price:
    Please sign in first.
    MSDS:
    Please sign in first.
    Packing:
    96T
    Polyclonal Antibody for Human Fibrillin-2
    Cat.:
    P8805Rb-m
    Price:
    Please sign in first.
    Packing:
    40ug/0.2ml
    Polyclonal Antibody for Human Fibrillin-2
    Cat.:
    P8805Rb-h
    Price:
    Please sign in first.
    Packing:
    40ug/0.2ml
    Monoclonal Antibody for Human Fibrillin-2
    Monoclonal Antibody for Human Fibrillin-2
    Protein for Human Fibrillin-2
    Protein for Human Fibrillin-2

    R&D Technical Data

    s

    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    Precision

    Intra-assay Precision (Precision within an assay):Three samples of known concentration were tested twenty times on one plate to assess intra-assay precision.

    Intra-Assay CV: ≤3.9%

    Inter-assay Precision (Precision between assays):Three samples of known concentration were tested in five separate assays to assess inter-assay precision.

    Inter-Assay CV: ≤7.7%

    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    Recovery

    Recovery was determined by spiking various levels of ACE into serum and plasma .

    Sample Type

    Average(%)

    Recovery Range(%)

    Serum

    99

    91-103

    Plasma

    98

    93-106

     

     

     

     

    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    Linearity

     

    The linearity of the kit was assayed by testing samples spiked with appropriate concentration of ACE and their serial dilutions. The results were demonstrated by the percentage of calculated concentration to the expected.  

     

    Sample

    1:2

    1:4

    1:8

    1:16

    serum(n=5)

    92-105%

    90-101%

    95-105%

    92-109%

    EDTA plasma(n=5)

    89-95%

    93105%

    89-94%

    90-106%

    heparin plasma(n=5)

    93-104%

     

    94-110%

    101-111%

    91-110%

     

    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    For more information, please refer to the manual,Or contact our technical support: tech@eiaab.com.
    References
    1. 1.
      "Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices."
      Zhang H. , Apfelroth S.D. , Hu W. , Davis E.C. , Sanguineti C. , Bonadio J. , Mecham R.P. , Ramirez F.
      J. Cell Biol.124:855-863(1994) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1);VARIANTS SER-594; GLY-1772; LEU-2266; LEU-2580 AND PRO-2771
    2. 2.
      "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T. , Suzuki Y. , Nishikawa T. , Otsuki T. , Sugiyama T. , Irie R. , Wakamatsu A. , Hayashi K. , Sato H. , Nagai K. , Kimura K. , Makita H. , Sekine M. , Obayashi M. , Nishi T. , Shibahara T. , Tanaka T. , Ishii S. , more...
      Nat. Genet.36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2)
      tissue: Placenta.
    3. 4.
      "Linkage of Marfan syndrome and a phenotypically related disorder to two different fibrillin genes."
      Lee B. , Godfrey M. , Vitale E. , Hori H. , Mattei M.-G. , Sarfarazi M. , Tsipouras P. , Ramirez F. , Hollister D.W.
      Nature352:330-334(1991) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 752-2912 (ISOFORM 2)
    4. 5.
      Totoki Y. , Toyoda A. , Takeda T. , Sakaki Y. , Tanaka A. , Yokoyama S. , Ohara O. , Nagase T. , Kikuno R.F.
      Submitted (2005-03) to the EMBL/GenBank/DDBJ databases
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 937-2912 (ISOFORM 1)
      tissue: Aortic endothelium.
    5. 6.
      "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
      Chen R. , Jiang X. , Sun D. , Han G. , Wang F. , Ye M. , Wang L. , Zou H.
      J. Proteome Res.8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-1112
      tissue: Liver.
    6. 7.
      "Fibrillin-2 (FBN2) mutations result in the Marfan-like disorder, congenital contractural arachnodactyly."
      Putnam E.A. , Zhang H. , Ramirez F. , Milewicz D.M.
      Nat. Genet.11:456-458(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS DA9 TYR-1253 AND SER-1434;VARIANT ILE-965
    7. 8.
      "Clustering of FBN2 mutations in patients with congenital contractural arachnodactyly indicates an important role of the domains encoded by exons 24 through 34 during human development."
      Park E.-S. , Putnam E.A. , Chitayat D. , Child A. , Milewicz D.M.
      Am. J. Med. Genet.78:350-355(1998) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS DA9 ASP-1057 AND THR-1093;VARIANTS SER-594; HIS-681; ILE-965; GLY-1772; LEU-2266; THR-2428 AND PRO-2771
    8. 9.
      "A single mutation that results in an Asp-to-His substitution and partial exon skipping in a family with congenital contractural arachnodactyly."
      Babcock D. , Gasner C. , Francke U. , Maslen C.
      Hum. Genet.103:22-28(1998) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT DA9 HIS-1115
    9. 10.
      "Two novel fibrillin-2 mutations in congenital contractural arachnodactyly."
      Belleh S. , Zhou G. , Wang M. , Der Kaloustian V.M. , Pagon R.A. , Godfrey M.
      Am. J. Med. Genet.92:7-12(2000) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS DA9 PHE-1142 AND TRP-1253
    10. 11.
      "Ten novel FBN2 mutations in congenital contractural arachnodactyly: delineation of the molecular pathogenesis and clinical phenotype."
      Gupta P.A. , Putnam E.A. , Carmical S.G. , Kaitila I. , Steinmann B. , Child A. , Danesino C. , Metcalfe K. , Berry S.A. , Chen E. , Delorme C.V. , Thong M.-K. , Ades L.C. , Milewicz D.M.
      Hum. Mutat.19:39-48(2002) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS DA9 ASP-1057; THR-1093; PHE-1142; CYS-1179; TYR-1198; ARG-1240; TRP-1253; TYR-1253; TRP-1257; ARG-1268 AND SER-1434;VARIANT ILE-965
    11. 12.
      "Comprehensive clinical and molecular assessment of 32 probands with congenital contractural arachnodactyly: report of 14 novel mutations and review of the literature."
      Callewaert B.L. , Loeys B.L. , Ficcadenti A. , Vermeer S. , Landgren M. , Kroes H.Y. , Yaron Y. , Pope M. , Foulds N. , Boute O. , Galan F. , Kingston H. , Van der Aa N. , Salcedo I. , Swinkels M.E. , Wallgren-Pettersson C. , Gabrielli O. , De Backer J. , more...
      Hum. Mutat.30:334-341(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS DA9 SER-754; SER-1091; HIS-1115; PRO-1122; ARG-1142; CYS-1146; PHE-1156; LYS-1161; PHE-1246; PHE-1384; TYR-1384; ASN-1408 AND ARG-1425
    12. 13.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT VAL-2062
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