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Hexosaminidase A 抗体 (Middle Region)

HEXA 适用: 人, 小鼠, 大鼠 WB, IHC 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN3044173
发货至: 中国
  • 抗原 See all Hexosaminidase A (HEXA) 抗体
    Hexosaminidase A (HEXA)
    抗原表位
    • 9
    • 8
    • 8
    • 7
    • 4
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 191-207, Middle Region
    适用
    • 59
    • 25
    • 14
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    人, 小鼠, 大鼠
    宿主
    • 50
    • 14
    克隆类型
    • 52
    • 12
    多克隆
    标记
    • 43
    • 4
    • 4
    • 3
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This Hexosaminidase A antibody is un-conjugated
    应用范围
    • 52
    • 33
    • 16
    • 8
    • 7
    • 7
    • 7
    • 4
    • 3
    • 1
    Western Blotting (WB), Immunohistochemistry (IHC)
    原理
    Anti-HEXA Antibody Picoband®
    序列
    DVMAYNKLNV FHWHLVD
    交叉反应 (详细)
    No cross-reactivity with other proteins
    产品特性
    Anti-HEXA Antibody (ABIN3044173). Tested in IHC, 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.
    纯化方法
    Immunogen affinity purified.
    免疫原
    A synthetic peptide corresponding to a sequence in the middle region of human HEXA, different from the related rat and mouse sequences by one amino acid.
    亚型
    IgG
    Top Product
    Discover our top product HEXA Primary Antibody
  • 应用备注
    Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human, Rat, Mouse
    Western blot, 0.1-0.5 μg/mL, Human, Rat, Mouse
    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.
    说明

    Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

    限制
    仅限研究用
  • 状态
    Lyophilized
    溶解方式
    Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
    浓度
    500 μg/mL
    缓冲液
    Each vial contains 5 mg BSA, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Thimerosal, 0.05 mg Sodium azide.
    储存液
    Thimerosal (Merthiolate), Sodium azide
    注意事项
    This product contains Thimerosal (Merthiolate) and Sodium azide: POISONOUS AND HAZARDOUS SUBSTANCES which should be handled by trained staff only.
    注意事项
    Avoid repeated freezing and thawing.
    储存条件
    4 °C,-20 °C
    储存方法
    Store 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 freeze-thaw cycles.
    有效期
    12 months
  • 抗原
    Hexosaminidase A (HEXA)
    别名
    HEXA (HEXA 产品)
    背景

    Synonyms: Beta-hexosaminidase subunit alpha,3.2.1.52,Beta-N-acetylhexosaminidase subunit alpha,Hexosaminidase subunit A,N-acetyl-beta-glucosaminidase subunit alpha,HEXA,

    Tissue Specificity: Expressed in various epithelial tissues. Abundantly expressed in liver and kidney, and to a lesser extent in lung. The tissue distribution of isoforms 1 and 1B is distinct. .

    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.

    Sequence Similarities: Belongs to the glycosyl hydrolase 20 family.

    分子量
    61 kDa
    UniProt
    P06865
    途径
    Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
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