GBE1 抗体 (AA 79-515)
Quick Overview for GBE1 抗体 (AA 79-515) (ABIN7602464)
抗原
See all GBE1 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 79-515
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原理
- Anti-GBE1 Antibody Picoband®
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产品特性
- Anti-GBE1 Antibody Picoband® (ABIN7602464). Tested in WB, ELISA applications. This antibody reacts with Human, Mouse, 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 GBE1 recombinant protein (Position: H79-R515). Human GBE1 shares 92.4% amino acid (aa) sequence identity with mouse GBE1.
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anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibody
GBE1 适用: 人 WB, IHC, IP, FACS, IHC (p) 宿主: 小鼠 Monoclonal 5B7 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibodyGBE1 适用: 人 WB, IF, IP, FACS 宿主: 小鼠 Monoclonal 4D9 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibodyGBE1 适用: 人 WB, IHC, IF, IP, FACS 宿主: 小鼠 Monoclonal 1D11 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 1-300) antibodyGBE1 适用: 人 WB, ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 1-702) antibodyGBE1 适用: 人 WB, IP 宿主: 兔 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 605-702) antibodyGBE1 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 1-702) antibodyGBE1 适用: 人 WB 宿主: 小鼠 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 527-556) antibodyGBE1 适用: 人, 小鼠 WB, IF 宿主: 兔 Polyclonal RB24029 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 534-564), (Middle Region) antibodyGBE1 适用: 人, 小鼠 WB, IF, EIA 宿主: 兔 Polyclonal unconjugated
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应用备注
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Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
ELISA, 0.1-0.5 μg/mL, -
1. Akman, H. O., Emmanuele, V., Kurt, Y. G., Kurt, B., Sheiko, T., DiMauro, S., Craigen, W. J. A novel mouse model that recapitulates adult-onset glycogenosis type 4. Hum. Molec. Genet. 24: 6801-6810, 2015. 2. Akman, H. O., Kakhlon, O., Coku, J., Peverelli, L., Rosenmann, H., Rozenstein-Tsalkovich, L., Turnbull, J., Meiner, V., Chama, L., Lerer, I., Shpitzen, S., Leitersdorf, E., Paradas, C., Wallace, M., Schiffmann, R., DiMauro, S., Lossos, A., Minassian, B. A. Deep intronic GBE1 mutation in manifesting heterozygous patients with adult polyglucosan body disease. JAMA Neurol. 72: 441-445, 2015. Note: Erratum: JAMA Neurol. 72: 481 only, 2015. 3. Alegria, A., Martins, E., Dias, M., Cunha, A., Cardoso, M. L., Maire, I. Glycogen storage disease type IV presenting as hydrops fetalis. J. Inherit. Metab. Dis. 22: 330-332, 1999. -
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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- GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
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别名
- GBE1
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背景
- 1,4-alpha-glucan-branching enzyme, also known as brancher enzyme or glycogen-branching enzyme is an enzyme that in humans is encoded by the GBE1 gene. The protein encoded by this gene is a glycogen branching enzyme that catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on the same or a neighboring glycogen chain. Branching of the chains is essential to increase the solubility of the glycogen molecule and, consequently, in reducing the osmotic pressure within cells. Highest level of this enzyme are found in liver and muscle. Mutations in this gene are associated with glycogen storage disease IV (also known as Andersen's disease).
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分子量
- 80 kDa
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基因ID
- 2632
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UniProt
- Q04446
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途径
- Cellular Glucan Metabolic Process
抗原
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