POMT1 抗体 (AA 224-747)
Quick Overview for POMT1 抗体 (AA 224-747) (ABIN7600716)
抗原
See all POMT1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 224-747
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原理
- Anti-POMT1 Antibody Picoband®
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交叉反应 (详细)
- No cross-reactivity with other proteins
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产品特性
- Anti-POMT1 Antibody Picoband® (ABIN7600716). Tested in WB, Flow Cytometry, ELISA applications. This antibody reacts with Human, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
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纯化方法
- Immunogen affinity purified.
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免疫原
- E.coli-derived human POMT1 recombinant protein (Position: H224-H747). Human POMT1 shares 81.1% and 80.9% amino acid (aa) sequence identity with mouse and rat POMT1, respectively.
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亚型
- IgG
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应用备注
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Western blot, 0.25-0.5 μg/mL, Human, Rat
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human
ELISA, 0.1-0.5 μg/mL
1. Balci, B., Uyanik, G., Dincer, P., Gross, C., Willer, T., Talim, B., Haliloglu, G., Kale, G., Hehr, U., Winkler, J., Topaloglu, H. An autosomal recessive limb girdle muscular dystrophy (LGMD2) with mild mental retardation is allelic to Walker-Warburg syndrome (WWS) caused by a mutation in the POMT1 gene. Neuromusc. Disord. 15: 271-275, 2005. 2. Bello, L., Melacini, P., Pezzani, R., D'Amico, A., Piva, L., Leonardi, E., Torella, A., Soraru, G., Palmieri, A., Smaniotto, G., Gavassini, B. F., Vianello, A., Nigro, V., Bertini, E., Angelini, C., Tosatto, S. C. E., Pegoraro, E. Cardiomyopathy in patients with POMT1-related congenital and limb-girdle muscular dystrophy. Europ. J. Hum. Genet. 20: 1234-1239, 2012. 3. Beltran-Valero de Bernabe, D., Currier, S., Steinbrecher, A., Celli, J., van Beusekom, E., van der Zwaag, B., Kayserili, H., Merlini, L., Chitayat, D., Dobyns, W. B., Cormand, B., Lehesjoki, A.-E., Cruces, J., Voit, T., Walsh, C. A., van Bokhoven, H., Brunner, H. G. Mutations in the O-mannosyltransferase gene POMT1 give rise to the severe neuronal migration disorder Walker-Warburg syndrome. Am. J. Hum. Genet. 71: 1033-1043, 2002. -
限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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浓度
- 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
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At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.
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- POMT1 (Protein-O-Mannosyltransferase 1 (POMT1))
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别名
- POMT1
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背景
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Synonyms: POMT1, Protein O-mannosyl-transferase 1, EC 2.4.1.109, Dolichyl-phosphate-mannose--protein mannosyltransferase 1
Background: Protein O-mannosyl-transferase 1 is an enzyme that in humans is encoded by the POMT1 gene. The protein encoded by this gene is an O-mannosyltransferase that requires interaction with the product of the POMT2 gene for enzymatic function. The encoded protein is found in the membrane of the endoplasmic reticulum. Defects in this gene are a cause of Walker-Warburg syndrome (WWS) and limb-girdle muscular dystrophy type 2K (LGMD2K). Several transcript variants encoding different isoforms have been found for this gene.
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分子量
- 75 kDa
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基因ID
- 10585
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UniProt
- Q9Y6A1
抗原
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