84-4416-73　FBN1 (Fibrillin 1, FBN, MASS, MFS1, OCTD, SGS, WMS, Marfan Syndrome) 200ul　363076

特徴

• Fibrillin-1 is a major component of the microfibrils that form a sheath surrounding the amorphous elastin.
• It is believed that the microfibrils are composed of end-to-end polymers of fibrillin.
• To date, 3 forms of fibrillin have been described.
• The fibrillin-1 protein was isolated by Engvall in 1986, and mutations in the gene have been linked to the Marfan syndrome.
• At present more than 100 different mutations have been described
• Trask et al.
• (1999) found that human FIB1 and FIB2 homodimerized via an N-terminal region and that the interaction was stabilized by disulphide bonds.
• Dimer formation occurred intracellularly, suggesting that the process of fibrillin aggregation initiates early after biosynthesis of the molecules.
• Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
• Other applications not tested.
• Recommended Dilution:ELISA: 1:100-1:5000Western Blot: 1:50-400Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100Immunocytochemistry: 1:50-500Optimal dilutions to be determined by the researcher
• AA Sequence:MSPILGYWKI KGLVQPTRLL LEYLEEKYEE HLYERDEGDK WRNKKFELGL EFPNLPYYID GDVKLTQSMA IIRYIADKHN MLGGCPKERA EISMLEGAVL DIRYGVSRIA YSKDFETLKV DFLSKLPEML KMFEDRLCHK TYLNGDHVTH PDFMLYDALD VVLYMDPMCL DAFPKLVCFK KRIEAIPQID KYLKSSKYIA WPLQGWQATF GGGDHPPKSD GSTSGSGHHH HHHSAGLVPR GSTAIGMKET AAAKFERQHM DSPDLGTLEV LFQGPLGS-VT DYCQLVRYLC QNGRCIPTPG SCRCECNKGF QLDLRGECID VDECEKNPCA GGECINNQGS YTCQCRAGYQ STLTRTECRD IDECLQNGRI CNNGRCINTD GSFHCVCNAG FHVTRDGKNC EDMDECSIRNMCLNGMCINE DGSFKCICKPGFQLASDGRY CKDINECETP GICMNGRCVN TDGSYRCECF PGLAVGLDGR VCVDTHMRST CYGGYKRGQC IKPLFGAVTK SECCCASTEY AFGEPCQPCP AQNSAEYQAL C
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.