Hexosaminidase A 抗体 (AA 135-179)
Quick Overview for Hexosaminidase A 抗体 (AA 135-179) (ABIN7599980)
抗原
See all Hexosaminidase A (HEXA) 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 135-179
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原理
- Anti-HEXA 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-HEXA Antibody Picoband® (ABIN7599980). Tested in ELISA, IF, ICC, WB 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 HEXA recombinant protein (Position: A135-H179).
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亚型
- IgG
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应用备注
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Western blot, 0.1-0.25 μg/mL, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
1. Akli, S., Chomel, J.-C., Lacorte, J.-M., Bachner, L., Poenaru, A., Poenaru, L. Ten novel mutations in the HEXA gene in non-Jewish Tay-Sachs patients. Hum. Molec. Genet. 2: 61-67, 1993. 2. Beutler, E., Kuhl, W., Comings, D. Hexosaminidase isozyme in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease). Am. J. Hum. Genet. 27: 628-638, 1975. 3. Chern, C. J., Beutler, E., Kuhl, W., Gilbert, F., Mellman, W. J., Croce, C. M. Characterization of heteropolymeric hexosaminidase A in human x mouse hybrid cells. Proc. Nat. Acad. Sci. 73: 3637-3640, 1976. -
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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- Hexosaminidase A (HEXA)
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别名
- HEXA
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背景
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Synonyms: Early growth response protein 1
Tissue Specificity: Detected in neutrophils (at protein level).
Background: HEXA, hexosaminidase A (alpha polypeptide), is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines. The HEXA gene encodes the alpha subunit of hexosaminidase A , a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit (amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.
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分子量
- 60 kDa
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基因ID
- 3073
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UniProt
- P06865
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途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
抗原
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