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Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein

级别和纯度:

≥95%(SDS-PAGE)

表达系统: HEK293
Accession #: P10253
蛋白标签: N-His
生物活性: Measured by its ability to release glucose from starch. The specific activity is >7,500 pmol/min/μg, as measured under the described conditions.
内毒素水平: <0.1 EU/μg

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货号 (SKU)	包装规格	是否现货
rp146225-10μg	10μg	现货
rp146225-50μg	50μg	现货
rp146225-100μg	100μg	现货
rp146225-1mg	1mg	期货

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点击查看蛋白信息: GAA

Accession#: P10253 (2) GAA (2) Gene ID: 2548 (2) Lysosomal alpha Glucosidase (1)

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基本描述

产品名称	Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein
别名	酸性葡糖苷酶α(GaA)重组蛋白
英文别名	Acid maltase \| Aglucosidase alfa \| EC 3.2.1.20 \| Acid Maltase \| Acid alpha-Glucosidase \| Aglucosidase alfa \| EC 3.2.1.20 \| GAA \| glucosidase, alpha \| acid \| LYAG \| Lysosomal alphaGlucosidase \| Lysosomal alpha-Glucosidase
规格或纯度	ActiBioPure™, 生物活性, 无动物源, Carrier Free, ≥95%(SDS-PAGE)
产品介绍	Essential for the degradation of glygogen to glucose in lysosomes.
生物活性	Measured by its ability to release glucose from starch. The specific activity is >7,500 pmol/min/μg, as measured under the described conditions.
内毒素水平	<0.1 EU/μg
表达系统	HEK293
种属	人(Human)
氨基酸	70-952 aa
序列	HHHHHHAHPGRPRAVPTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHVHSRAPSPLYSVEFSEEPFGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPLMLSTSWTRITLWNRDLAPTPGANLYGSHPFYLALEDGGSAHGVFLLNSNAMDVVLQPSPALSWRSTGGILDVYIFLGPEPKSVVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSGPAGSYRPYDEGLRRGVFITNETGQPLIGKVWPGSTAFPDFTNPTALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIRGSEDGCPNNELENPPYVPGVVGGTLQAATICASSHQFLSTHYNLHNLYGLTEAIASHRALVKARGTRPFVISRSTFAGHGRYAGHWTGDVWSSWEQLASSVPEILQFNLLGVPLVGADVCGFLGNTSEELCVRWTQLGAFYPFMRNHNSLLSLPQEPYSFSEPAQQAMRKALTLRYALLPHLYTLFHQAHVAGETVARPLFLEFPKDSSTWTVDHQLLWGEALLITPVLQAGKAEVTGYFPLGTWYDLQTVPVEALGSLPPPPAAPREPAIHSEGQWVTLPAPLDTINVHLRAGYIIPLQGPGLTTTESRQQPMALAVALTKGGEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC
蛋白标签	N-His
无载体	Yes
无动物源	Yes
Accession #	P10253
来源	重组表达
预测分子量	99 kDa
SDS-PAGE	95-105 kDa, under reducing conditions

储存与运输

物理形态	冻干(Lyophilized)
储存缓冲液	Lyophilized from 10mM PBS, 5% Trehalose, 5% Mannitol, pH 7.4
复溶	Reconstitute in sterile water to a concentration of 0.1-0.5 mg/ml.
储存温度	-20°C储存,避免反复冻融
运输条件	超低温冰袋运输
稳定性与储存	在 -20~-80℃ 下可保存 1 年以上。复溶后建议分装。避免冷冻/解冻循环。
分子类型	蛋白质

图片

SDS-PAGE: Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein (rp146225)

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein (rp146225) - SDS-PAGE
2 μg/lane of Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein was resolved with SDS-PAGE under reducing (R) and non-reducing (N) conditions and visualized by Coomassie® Blue staining, showing the band at 105 - 120 kDa under reducing conditions and 260 kDa under non-reducing conditions.

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找到3个结果

批号(Lot Number)	证书类型	货号
ZJ25F0724748	分析证书	rp146225
ZJ25F0724747	分析证书	rp146225
ZJ25F0724746	分析证书	rp146225

引用文献

1. Asano N, Ishii S, Kizu H, Ikeda K, Yasuda K, Kato A, Martin OR, Fan JQ. (2000) In vitro inhibition and intracellular enhancement of lysosomal alpha-galactosidase A activity in Fabry lymphoblasts by 1-deoxygalactonojirimycin and its derivatives.. Eur J Biochem, 267 (13): (4179-86). [PMID:10866822]
2. Marugan JJ, Zheng W, Motabar O, Southall N, Goldin E, Sidransky E, Aungst RA, Liu K, Sadhukhan SK, Austin CP.. (2010) Evaluation of 2-thioxo-2,3,5,6,7,8-hexahydropyrimido[4,5-d]pyrimidin-4(1H)-one analogues as GAA activators.. Eur J Med Chem, 45 (5): (1880-1897). [PMID:20206419] [10.1016/j.ejmech.2010.01.027]
3. Castilla J, Rísquez R, Cruz D, Higaki K, Nanba E, Ohno K, Suzuki Y, Díaz Y, Ortiz Mellet C, García Fernández JM, Castillón S.. (2012) Conformationally-locked N-glycosides with selective β-glucosidase inhibitory activity: identification of a new non-iminosugar-type pharmacological chaperone for Gaucher disease.. J Med Chem, 55 (15): (6857-6865). [PMID:22762530] [10.1021/jm3006178]
4. Patnaik S, Zheng W, Choi JH, Motabar O, Southall N, Westbroek W, Lea WA, Velayati A, Goldin E, Sidransky E, Leister W, Marugan JJ.. (2012) Discovery, structure-activity relationship, and biological evaluation of noninhibitory small molecule chaperones of glucocerebrosidase.. J Med Chem, 55 (12): (5734-5748). [PMID:22646221] [10.1021/jm300063b]
5. Xiao J, Westbroek W, Motabar O, Lea WA, Hu X, Velayati A, Zheng W, Southall N, Gustafson AM, Goldin E, Sidransky E, Liu K, Simeonov A, Tamargo RJ, Ribes A, Matalonga L, Ferrer M, Marugan JJ.. (2012) Discovery of a novel noniminosugar acid α glucosidase chaperone series.. J Med Chem, 55 (17): (7546-7559). [PMID:22834902] [10.1021/jm3005543]
6. Kato A, Hayashi E, Miyauchi S, Adachi I, Imahori T, Natori Y, Yoshimura Y, Nash RJ, Shimaoka H, Nakagome I, Koseki J, Hirono S, Takahata H.. (2012) α-1-C-butyl-1,4-dideoxy-1,4-imino-l-arabinitol as a second-generation iminosugar-based oral α-glucosidase inhibitor for improving postprandial hyperglycemia.. J Med Chem, 55 (23): (10347-10362). [PMID:23106358] [10.1021/jm301304e]
7. Cheng WC, Weng CY, Yun WY, Chang SY, Lin YC, Tsai FJ, Huang FY, Chen YR.. (2013) Rapid modifications of N-substitution in iminosugars: development of new β-glucocerebrosidase inhibitors and pharmacological chaperones for Gaucher disease.. Bioorg Med Chem, 21 (17): (5021-5028). [PMID:23880081] [10.1016/j.bmc.2013.06.054]
8. Castilla J, Rísquez R, Higaki K, Nanba E, Ohno K, Suzuki Y, Díaz Y, Ortiz Mellet C, García Fernández JM, Castillón S.. (2015) Conformationally-locked N-glycosides: exploiting long-range non-glycone interactions in the design of pharmacological chaperones for Gaucher disease.. Eur J Med Chem, 90 (258-266). [PMID:25461326] [10.1016/j.ejmech.2014.11.002]
9. Zheng J, Chen L, Schwake M, Silverman RB, Krainc D.. (2016) Design and Synthesis of Potent Quinazolines as Selective β-Glucocerebrosidase Modulators.. J Med Chem, 59 (18): (8508-8520). [PMID:27598312] [10.1021/acs.jmedchem.6b00930]
10. Cheng WC, Wang JH, Li HY, Lu SJ, Hu JM, Yun WY, Chiu CH, Yang WB, Chien YH, Hwu WL.. (2016) Bioevaluation of sixteen ADMDP stereoisomers toward alpha-galactosidase A: Development of a new pharmacological chaperone for the treatment of Fabry disease and potential enhancement of enzyme replacement therapy efficiency.. Eur J Med Chem, 123 (14-20). [PMID:27474919] [10.1016/j.ejmech.2016.07.025]
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13. Li ZP, Song YH, Uddin Z, Wang Y, Park KH.. (2018) Inhibition of protein tyrosine phosphatase 1B (PTP1B) and α-glucosidase by xanthones from Cratoxylum cochinchinense, and their kinetic characterization.. Bioorg Med Chem, 26 (3): (737-746). [PMID:29306546] [10.1016/j.bmc.2017.12.043]
14. Zhang XQ, Mou XF, Mao N, Hao JJ, Liu M, Zheng JY, Wang CY, Gu YC, Shao CL.. (2018) Design, semisynthesis, α-glucosidase inhibitory, cytotoxic, and antibacterial activities of p-terphenyl derivatives.. Eur J Med Chem, 146 (232-244). [PMID:29407953] [10.1016/j.ejmech.2018.01.057]
15. Luthra T, Naga Lalitha K, Agarwal R, Uma A, Sen S.. (2018) Design, synthesis and in vitro study of densely functionalized oxindoles as potent α-glucosidase inhibitors.. Bioorg Med Chem, 26 (18): (4996-5005). [PMID:30153956] [10.1016/j.bmc.2018.08.022]
16. Dhameja M, Gupta P.. (2019) Synthetic heterocyclic candidates as promising α-glucosidase inhibitors: An overview.. Eur J Med Chem, 176 (343-377). [PMID:31112894] [10.1016/j.ejmech.2019.04.025]
17. Spasov AA, Babkov DA, Osipov DV, Klochkov VG, Prilepskaya DR, Demidov MR, Osyanin VA, Klimochkin YN.. (2019) Synthesis, in vitro and in vivo evaluation of 2-aryl-4H-chromene and 3-aryl-1H-benzo[f]chromene derivatives as novel α-glucosidase inhibitors.. Bioorg Med Chem Lett, 29 (1): (119-123). [PMID:30340897] [10.1016/j.bmcl.2018.10.018]
18. Ye GJ, Lan T, Huang ZX, Cheng XN, Cai CY, Ding SM, Xie ML, Wang B.. (2019) Design and synthesis of novel xanthone-triazole derivatives as potential antidiabetic agents: α-Glucosidase inhibition and glucose uptake promotion.. Eur J Med Chem, 177 (362-373). [PMID:31158750] [10.1016/j.ejmech.2019.05.045]
19. Choi RY, Nam SJ, Ham JR, Lee HI, Yee ST, Kang KY, Seo KI, Lee JH, Kim MJ, Lee MK.. (2016) Anti-adipogenic and anti-diabetic effects of cis-3',4'-diisovalerylkhellactone isolated from Peucedanum japonicum Thunb leaves in vitro.. Bioorg Med Chem Lett, 26 (19): (4655-4660). [PMID:27575482] [10.1016/j.bmcl.2016.08.056]
20. Zhong, N N, Martiniuk, F F, Tzall, S S and Hirschhorn, R R.. (1991) Identification of a missense mutation in one allele of a patient with Pompe disease, and use of endonuclease digestion of PCR-amplified RNA to demonstrate lack of mRNA expression from the second allele.. American journal of human genetics, [PMID:1652892]
21. Martiniuk, F F and 6 more authors.. (1991) Identification of a missense mutation in an adult-onset patient with glycogenosis type II expressing only one allele.. DNA and cell biology, [PMID:1684505]
22. Hermans, M M MM, Kroos, M A MA, van Beeumen, J J, Oostra, B A BA and Reuser, A J AJ.. (1991) Human lysosomal alpha-glucosidase. Characterization of the catalytic site.. The Journal of biological chemistry, (25): [PMID:1856189]
23. Hermans, M M MM and 5 more authors.. (1991) Identification of a point mutation in the human lysosomal alpha-glucosidase gene causing infantile glycogenosis type II.. Biochemical and biophysical research communications, (16): [PMID:1898413]
24. Martiniuk, F F, Mehler, M M, Tzall, S S, Meredith, G G and Hirschhorn, R R.. (1990) Sequence of the cDNA and 5'-flanking region for human acid alpha-glucosidase, detection of an intron in the 5' untranslated leader sequence, definition of 18-bp polymorphisms, and differences with previous cDNA and amino acid sequences.. DNA and cell biology, [PMID:2111708]
25. Martiniuk, F F, Bodkin, M M, Tzall, S S and Hirschhorn, R R.. (1990) Identification of the base-pair substitution responsible for a human acid alpha glucosidase allele with lower "affinity" for glycogen (GAA 2) and transient gene expression in deficient cells.. American journal of human genetics, [PMID:2203258]
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27. Hoefsloot, L H LH and 5 more authors.. (1988) Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex.. The EMBO journal, [PMID:3049072]
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30. Boerkoel, C F CF and 6 more authors.. (1995) Leaky splicing mutation in the acid maltase gene is associated with delayed onset of glycogenosis type II.. American journal of human genetics, [PMID:7717400]
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32. Hermans, M M MM and 9 more authors.. (1994) The effect of a single base pair deletion (delta T525) and a C1634T missense mutation (pro545leu) on the expression of lysosomal alpha-glucosidase in patients with glycogen storage disease type II.. Human molecular genetics, [PMID:7881422]
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34. Hermans, M M MM and 6 more authors.. (1993) The conservative substitution Asp-645-->Glu in lysosomal alpha-glucosidase affects transport and phosphorylation of the enzyme in an adult patient with glycogen-storage disease type II.. The Biochemical journal, (1): [PMID:8094613]
35. Hermans, M M MM, Kroos, M A MA, de Graaff, E E, Oostra, B A BA and Reuser, A J AJ.. (1993) Two mutations affecting the transport and maturation of lysosomal alpha-glucosidase in an adult case of glycogen storage disease type II.. Human mutation, [PMID:8401535]
36. Hermans, M M MM, Wisselaar, H A HA, Kroos, M A MA, Oostra, B A BA and Reuser, A J AJ.. (1993) Human lysosomal alpha-glucosidase: functional characterization of the glycosylation sites.. The Biochemical journal, (1): [PMID:8435067]
37. Hermans, M M MM, Svetkey, L P LP, Oostra, B A BA, Chen, Y T YT and Reuser, A J AJ.. (1993) The loss of a polymorphic glycosylation site caused by Thr-927-->Ile is linked to a second polymorphic Val-816-->Ile substitution in lysosomal alpha-glucosidase of American blacks.. Genomics, [PMID:8486380]
38. Tsunoda, H H and 5 more authors.. (1996) Acid alpha-glucosidase deficiency: identification and expression of a missense mutation (S529V) in a Japanese adult phenotype.. Human genetics, [PMID:8834250]
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40. Hermans, M M MM and 5 more authors.. (1998) Glycogen Storage Disease type II: genetic and biochemical analysis of novel mutations in infantile patients from Turkish ancestry.. Human mutation, [PMID:9521422]
41. Huie, M L ML and 10 more authors.. (1998) Glycogen storage disease type II: identification of four novel missense mutations (D645N, G648S, R672W, R672Q) and two insertions/deletions in the acid alpha-glucosidase locus of patients of differing phenotype.. Biochemical and biophysical research communications, (27): [PMID:9535769]
42. Kroos, M A MA, van Leenen, D D, Verbiest, J J, Reuser, A J AJ and Hermans, M M MM.. (1998) Glycogen storage disease type II: identification of a dinucleotide deletion and a common missense mutation in the lysosomal alpha-glucosidase gene.. Clinical genetics, [PMID:9660056]
43. Raben, N N, Lee, E E, Lee, L L, Hirschhorn, R R and Plotz, P H PH.. (1999) Novel mutations in African American patients with glycogen storage disease Type II. Mutations in brief no. 209. Online.. Human mutation, [PMID:10189220]
44. Beesley, C E CE, Child, A H AH and Yacoub, M H MH.. (1998) The identification of five novel mutations in the lysosomal acid a-(1-4) glucosidase gene from patients with glycogen storage disease type II. Mutations in brief no. 134. Online.. Human mutation, [PMID:10206684]
45. Ko, T M TM and 6 more authors.. (1999) Molecular genetic study of Pompe disease in Chinese patients in Taiwan.. Human mutation, [PMID:10338092]
46. Vorgerd, M M, Burwinkel, B B, Reichmann, H H, Malin, J P JP and Kilimann, M W MW.. (1998) Adult-onset glycogen storage disease type II: phenotypic and allelic heterogeneity in German patients.. Neurogenetics, [PMID:10737124]
47. Laforêt, P P and 6 more authors.. (2000) Juvenile and adult-onset acid maltase deficiency in France: genotype-phenotype correlation.. Neurology, (24): [PMID:11071489]
48. Fernandez-Hojas, Roberto R and 8 more authors.. (2002) Identification of six novel mutations in the acid alpha-glucosidase gene in three Spanish patients with infantile onset glycogen storage disease type II (Pompe disease).. Neuromuscular disorders : NMD, [PMID:11738358]
49. Lam, C W CW and 8 more authors.. (2003) Juvenile-onset glycogen storage disease type II with novel mutations in acid alpha-glucosidase gene.. Neurology, (25): [PMID:12601120]
50. Zhang, Hui H, Li, Xiao-Jun XJ, Martin, Daniel B DB and Aebersold, Ruedi R.. (2003) Identification and quantification of N-linked glycoproteins using hydrazide chemistry, stable isotope labeling and mass spectrometry.. Nature biotechnology, [PMID:12754519]
51. Pittis, Maria Gabriela MG and 7 more authors.. (2003) Identification of four novel mutations in the alpha glucosidase gene in five Italian patients with infantile onset glycogen storage disease type II.. American journal of medical genetics. Part A, (1): [PMID:12923862]
52. Pipo, Judy R JR and 10 more authors.. (2003) New GAA mutations in Japanese patients with GSDII (Pompe disease).. Pediatric neurology, [PMID:14643388]
53. Hermans, Monique M P MM and 14 more authors.. (2004) Twenty-two novel mutations in the lysosomal alpha-glucosidase gene (GAA) underscore the genotype-phenotype correlation in glycogen storage disease type II.. Human mutation, [PMID:14695532]
54. Kroos, Marian A MA and 6 more authors.. (2004) A case of childhood Pompe disease demonstrating phenotypic variability of p.Asp645Asn.. Neuromuscular disorders : NMD, [PMID:15145338]
55. Anneser, J M H JM, Pongratz, D E DE, Podskarbi, T T, Shin, Y S YS and Schoser, B G H BG.. (2005) Mutations in the acid alpha-glucosidase gene (M. Pompe) in a patient with an unusual phenotype.. Neurology, (25): [PMID:15668445]
56. Liu, Tao T and 6 more authors.. Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry.. Journal of proteome research, [PMID:16335952]
57. Amartino, H H and 6 more authors.. (2006) Two clinical forms of glycogen-storage disease type II in two generations of the same family.. Clinical genetics, [PMID:16433701]
58. Zody, Michael C MC and 73 more authors.. (2006) DNA sequence of human chromosome 17 and analysis of rearrangement in the human lineage.. Nature, (20): [PMID:16625196]
59. Gelb, Michael H MH, Turecek, Frantisek F, Scott, C Ron CR and Chamoles, Nestor A NA.. Direct multiplex assay of enzymes in dried blood spots by tandem mass spectrometry for the newborn screening of lysosomal storage disorders.. Journal of inherited metabolic disease, [PMID:16763908]
60. Dou, Wei W and 6 more authors.. (2006) A novel missense mutation in the acid alpha-glucosidase gene causing the classic infantile form of Pompe disease.. Clinica chimica acta; international journal of clinical chemistry, [PMID:16782080]
61. Montalvo, A L E AL and 12 more authors.. (2006) Mutation profile of the GAA gene in 40 Italian patients with late onset glycogen storage disease type II.. Human mutation, [PMID:16917947]
62. Schröder, Bernd B and 8 more authors.. (2007) Integral and associated lysosomal membrane proteins.. Traffic (Copenhagen, Denmark), [PMID:17897319]
63. Kroos, Marian M and 8 more authors.. (2008) Update of the Pompe disease mutation database with 107 sequence variants and a format for severity rating.. Human mutation, [PMID:18425781]
64. Pittis, M G MG and 15 more authors.. (2008) Molecular and functional characterization of eight novel GAA mutations in Italian infants with Pompe disease.. Human mutation, [PMID:18429042]
65. Oba-Shinjo, Sueli M SM and 24 more authors.. (2009) Pompe disease in a Brazilian series: clinical and molecular analyses with identification of nine new mutations.. Journal of neurology, [PMID:19588081]
66. Fukaya, Nanae N and 6 more authors.. (2009) The alpha-glucosidase inhibitor miglitol delays the development of diabetes and dysfunctional insulin secretion in pancreatic beta-cells in OLETF rats.. European journal of pharmacology, (10): [PMID:19818342]
67. Labrousse, Paul P and 7 more authors.. (2010) Genetic heterozygosity and pseudodeficiency in the Pompe disease newborn screening pilot program.. Molecular genetics and metabolism, [PMID:20080426]
68. Alcántara-Ortigoza, Miguel Angel MA and 6 more authors.. (2010) Screening of late-onset Pompe disease in a sample of Mexican patients with myopathies of unknown etiology: identification of a novel mutation in the acid alpha-glucosidase gene.. Journal of child neurology, [PMID:20350966]
69. Kroos, Marian M and 7 more authors.. (2012) Update of the pompe disease mutation database with 60 novel GAA sequence variants and additional studies on the functional effect of 34 previously reported variants.. Human mutation, [PMID:22644586]
70. Herzog, Andreas A and 11 more authors.. (2012) A cross-sectional single-centre study on the spectrum of Pompe disease, German patients: molecular analysis of the GAA gene, manifestation and genotype-phenotype correlations.. Orphanet journal of rare diseases, (7): [PMID:22676651]
71. Turaça, Lauro Thiago LT and 11 more authors.. (2015) Novel GAA mutations in patients with Pompe disease.. Gene, (25): [PMID:25681614]
72. Roig-Zamboni, Véronique V and 8 more authors.. (2017) Structure of human lysosomal acid α-glucosidase-a guide for the treatment of Pompe disease.. Nature communications, (24): [PMID:29061980]

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