Alpha-L-Iduronidase (IDUA) Human Recombinant Protein

MBS717919 is a recombinant protein targeting IDUA. Also known as Recombinant Human Alpha-L-iduronidase (IDUA). Host: E Coli or Yeast or Baculovirus or Mammalian Cell.

Human alpha-L-iduronidase cDNA isolation and expression.Scott H.S., Anson D.S., Orsborn A.M., Nelson P.V., Clements P.R., Morris C.P., Hopwood J.J.Proc. Natl. Acad. Sci. U.S.A. 88:9695-9699(1991) Structure and sequence of the human alpha-L-iduronidase gene.Scott H.S., Guo X.H., Hopwood J.J., Morris C.P.Genomics 13:1311-1313(1992) 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., Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.Nat. Genet. 36:40-45(2004) Generation and annotation of the DNA sequences of human chromosomes 2 and 4.Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P., Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C., Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L., Du H., Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A., Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J., Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M., Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T., Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S., Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K., McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C., Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S., Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C., Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M., Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C., Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J., Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E., Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X., Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M., Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C., Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S., Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H., Wilson R.K.Nature 434:724-731(2005) Molecular genetics of mucopolysaccharidosis type I diagnostic, clinical, and biological implications.Scott H.S., Bunge S., Gal A., Clarke L.A., Morris C.P., Hopwood J.J.Hum. Mutat. 6:288-302(1995) 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) Insights into mucopolysaccharidosis I from the structure and action of alpha-L-iduronidase.Bie H., Yin J., He X., Kermode A.R., Goddard-Borger E.D., Withers S.G., James M.N.Nat. Chem. Biol. 9:739-745(2013) Human alpha-L-iduronidase uses its own N-glycan as a substrate-binding and catalytic module.Maita N., Tsukimura T., Taniguchi T., Saito S., Ohno K., Taniguchi H., Sakuraba H.Proc. Natl. Acad. Sci. U.S.A. 110:14628-14633(2013) Mutation analysis of 19 North American mucopolysaccharidosis type I patients identification of two additional frequent mutations.Clarke L.A., Nelson P.V., Warrington C.L., Morris C.P., Hopwood J.J., Scott H.S.Hum. Mutat. 3:275-282(1994) Two novel mutations causing mucopolysaccharidosis type I detected by single strand conformational analysis of the alpha-L-iduronidase gene.Clark L.A., Scott H.S.Hum. Mol. Genet. 2:1311-1312(1993) Identification of mutations in the alpha-L-iduronidase gene (IDUA) that cause Hurler and Scheie syndromes.Scott H.S., Litjens T., Nelson P.V., Thompson P.R., Brooks D.A., Hopwood J.J., Morris C.P.Am. J. Hum. Genet. 53:973-986(1993) Molecular analysis of Hurler syndrome in Druze and Muslim Arab patients in Israel multiple allelic mutations of the IDUA gene in a small geographic area.Bach G., Moskowitz S.M., Tieu P.T., Matynia A., Neufeld E.F.Am. J. Hum. Genet. 53:330-338(1993) Alpha-L-iduronidase mutations (Q70X and P533R) associate with a severe Hurler phenotype.Scott H.S., Litjens T., Nelson P.V., Brooks D.A., Hopwood J.J., Morris C.P.Hum. Mutat. 1:333-339(1992) Mucopolysaccharidosis type I identification of 8 novel mutations and determination of the frequency of the two common alpha-L-iduronidase mutations (W402X and Q70X) among European patients.Bunge S., Kleijer W.J., Steglich C., Beck M., Zuther C., Morris C.P., Schwinger E., Hopwood J.J., Scott H.S., Gal A.Hum. Mol. Genet. 3:861-866(1994) PCR detection of two RFLPs in exon I of the alpha-L-iduronidase (IDUA) gene.Scott H.S., Litjens T., Hopwood J.J., Morris C.P.Hum. Genet. 90:327-327(1992) Multiple polymorphisms within the alpha-L-iduronidase gene (IDUA) implications for a role in modification of MPS-I disease phenotype.Scott H.S., Nelson P.V., Litjens T., Hopwood J.J., Morris C.P.Hum. Mol. Genet. 2:1471-1473(1993) Four novel mutations underlying mild or intermediate forms of alpha-L-iduronidase deficiency (MPS IS and MPS IH/S) .Tieu P.T., Bach G., Matynia A., Hwang M., Neufeld E.F.Hum. Mutat. 6:55-59(1995) Mucopolysaccharidosis type I identification of 13 novel mutations of the alpha-L-iduronidase gene.Bunge S., Kleijer W.J., Steglich C., Beck M., Schwinger E., Gal A.Hum. Mutat. 6:91-94(1995) Molecular genetic defect underlying alpha-L-iduronidase pseudodeficiency.Aronovich E.L., Pan D., Whitley C.B.Am. J. Hum. Genet. 58:75-85(1996) A novel missense mutation in the human IDUA gene associated with a severe Hurler's phenotype.Bartholomew D.W., McClellan J.M.Hum. Mutat. 12:291-291(1998) Mucopolysaccharidosis type I characterization of novel mutations affecting alpha-L-iduronidase activity.Lee-Chen G.J., Lin S.P., Tang Y.F., Chin Y.W.Clin. Genet. 56:66-70(1999) Identification and characterization of -3c-g acceptor splice site mutation in human alpha-L-iduronidase associated with mucopolysaccharidosis type IH/S.Teng Y.N., Wang T.R., Hwu W.L., Lin S.P., Lee-Chen G.J.Clin. Genet. 57:131-136(2000) Identification and characterization of 13 new mutations in mucopolysaccharidosis type I patients.Matte U., Yogalingam G., Brooks D., Leistner S., Schwartz I., Lima L., Norato D.Y., Brum J.M., Beesley C., Winchester B., Giugliani R., Hopwood J.J.Mol. Genet. Metab. 78:37-43(2003) Identification and molecular characterization of alpha-L-iduronidase mutations present in mucopolysaccharidosis type I patients undergoing enzyme replacement therapy.Yogalingam G., Guo X.H., Muller V.J., Brooks D.A., Clements P.R., Kakkis E.D., Hopwood J.J.Hum. Mutat. 24:199-207(2004) Mucopolysaccharidosis type I in 21 Czech and Slovak patients mutation analysis suggests a functional importance of C-terminus of the IDUA protein.Vazna A., Beesley C., Berna L., Stolnaja L., Myskova H., Bouckova M., Vlaskova H., Poupetova H., Zeman J., Magner M., Hlavata A., Winchester B., Hrebicek M., Dvorakova L.Am. J. Med. Genet. A 149A:965-974(2009) IDUA mutational profiling of a cohort of 102 European patients with mucopolysaccharidosis type I identification and characterization of 35 novel alpha-L-iduronidase (IDUA) alleles.Bertola F., Filocamo M., Casati G., Mort M., Rosano C., Tylki-Szymanska A., Tuysuz B., Gabrielli O., Grossi S., Scarpa M., Parenti G., Antuzzi D., Dalmau J., Di Rocco M., Vici C.D., Okur I., Rosell J., Rovelli A., Furlan F., Rigoldi M., Biondi A., Cooper D.N., Parini R.Hum. Mutat. 32:E2189-E2210(2011) p.L18P a novel IDUA mutation that causes a distinct attenuated phenotype in mucopolysaccharidosis type I patients.Pasqualim G., Ribeiro M.G., da Fonseca G.G., Szlago M., Schenone A., Lemes A., Rojas M.V., Matte U., Giugliani R.Clin. Genet. 0:0-0(2014) +Additional computationally mapped references.<p>Provides general information on the entry.

Small volumes of IDUA recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.