Alpha-L-iduronidase (IDUA) Recombinant Protein | IDUA recombinant protein

Recombinant Human Alpha-L-iduronidase (IDUA)

Cat. Num. AAA15934

• Gene Names: IDUA; IDA; MPS1
• Purity: Greater or equal to 85% purity as determined by SDS-PAGE.
• Synonyms: Alpha-L-iduronidase (IDUA); N/A; Recombinant Human Alpha-L-iduronidase (IDUA); IDUA recombinant protein

• Host: E Coli or Yeast or Baculovirus or Mammalian Cell
• Purity/Purification: Greater or equal to 85% purity as determined by SDS-PAGE.
• Form/Format: Lyophilized or liquid (Format to be determined during the manufacturing process)
• Sequence Positions: 28-653aa; Full Length of Mature Protein
• Sequence: APHLVHVDAARALWPLRRFWRSTGFCPPLPHSQADQYVLSWDQQLNLAYVGAVPHRGIKQVRTHWLLELVTTRGSTGRGLSYNFTHLDGYLDLLRENQLLPGFELMGSASGHFTDFEDKQQVFEWKDLVSSLARRYIGRYGLAHVSKWNFETWNEPDHHDFDNVSMTMQGFLNYYDACSEGLRAASPALRLGGPGDSFHTPPRSPLSWGLLRHCHDGTNFFTGEAGVRLDYISLHRKGARSSISILEQEKVVAQQIRQLFPKFADTPIYNDEADPLVGWSLPQPWRADVTYAAMVVKVIAQHQNLLLANTTSAFPYALLSNDNAFLSYHPHPFAQRTLTARFQVNNTRPPHVQLLRKPVLTAMGLLALLDEEQLWAEVSQAGTVLDSNHTVGVLASAHRPQGPADAWRAAVLIYASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGLCSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHVCARPEKPPGQVTRLRALPLTQGQLVLVWSDEHVGSKCLWTYEIQFSQDGKAYTPVSRKPSTFNLFVFSPDTGAVSGSYRVRALDYWARPGPFSDPVPYLEVPVPRGPPSPGNP

• Preparation and Storage: Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C.

SDS-PAGE

Product Categories/Family for IDUA recombinant protein

Metabolism

References

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.

NCBI and Uniprot Product Information

• NCBI GI #: 110611239
• NCBI GeneID: 3425
• NCBI Accession #: NP_000194.2
• NCBI GenBank Nucleotide #: NM_000203.4
• UniProt Accession #: P35475; B3KWK6
• Molecular Weight: 71.9 kDa
• NCBI Official Full Name: alpha-L-iduronidase
• NCBI Official Synonym Full Names: iduronidase, alpha-L-
• NCBI Official Symbol: IDUA
• NCBI Official Synonym Symbols: IDA; MPS1
• NCBI Protein Information: alpha-L-iduronidase
• UniProt Protein Name: Alpha-L-iduronidase
• UniProt Gene Name: IDUA
• UniProt Entry Name: IDUA_HUMAN