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GBA2 抗体 (AA 17-869)

The 兔 多克隆 anti-GBA2 antibody is suitable to detect GBA2 in samples from 人, 小鼠 和 大鼠. It has been validated for WB, ELISA 和 FACS.
产品编号 ABIN7871244
发货至: 中国
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Quick Overview for GBA2 抗体 (AA 17-869) (ABIN7871244)

抗原

See all GBA2 抗体
GBA2 (Glucosidase, beta (Bile Acid) 2 (GBA2))

适用

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人, 小鼠, 大鼠

宿主

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克隆类型

  • 44
多克隆

标记

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This GBA2 antibody is un-conjugated

应用范围

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Western Blotting (WB), ELISA, Flow Cytometry (FACS)
  • 抗原表位

    • 15
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    AA 17-869

    原理

    GBA2 Antibody / Non-lysosomal glucosylceramidase

    纯化方法

    Immunogen affinity purified

    免疫原

    E.coli-derived human GBA2 recombinant protein (Position: E17-Q869) was used as the immunogen for the GBA2 antibody.

    亚型

    IgG
  • 应用备注

    Optimal dilution of the GBA2 antibody should be determined by the researcher.

    限制

    仅限研究用
  • 状态

    Lyophilized

    溶解方式

    Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

    缓冲液

    Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

    储存条件

    4 °C,-20 °C

    储存方法

    After reconstitution, the GBA2 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
  • 抗原

    GBA2 (Glucosidase, beta (Bile Acid) 2 (GBA2))

    别名

    GBA2

    背景

    GBA2 antibody detects Non-lysosomal glucosylceramidase, a cytoplasmic enzyme responsible for the hydrolysis of glucosylceramide to glucose and ceramide outside of lysosomes. The UniProt recommended name is Non-lysosomal glucosylceramidase (GBA2). This enzyme participates in sphingolipid metabolism and maintains the balance of glycosphingolipids that influence membrane structure, signaling, and lipid homeostasis.

    Functionally, GBA2 antibody identifies a 927-amino-acid membrane-associated protein localized to the endoplasmic reticulum and Golgi apparatus. GBA2 catalyzes the removal of glucose from glucosylceramide, complementing the lysosomal enzyme GBA1 but functioning in non-lysosomal compartments. By regulating ceramide and glucosylceramide levels, GBA2 affects cell differentiation, apoptosis, and neuronal lipid metabolism.

    The GBA2 gene is located on chromosome 9p13.3 and is highly expressed in brain, liver, testis, and kidney. Its activity is essential for maintaining sphingolipid turnover and membrane lipid composition, particularly in neuronal and reproductive tissues. GBA2 functions as a key metabolic enzyme in glycosphingolipid homeostasis and intracellular lipid transport.

    Pathologically, mutations in GBA2 cause hereditary spastic paraplegia type 46 and autosomal recessive cerebellar ataxia, disorders characterized by impaired motor function and cerebellar degeneration. Loss of GBA2 activity leads to accumulation of glucosylceramide, resulting in disrupted lipid trafficking and neuronal dysfunction. Research using GBA2 antibody supports studies in lipid metabolism, neurobiology, and metabolic disease mechanisms.

    GBA2 antibody is validated for western blotting, immunohistochemistry, and immunofluorescence to detect glucosylceramidase enzymes. NSJ Bioreagents provides GBA2 antibody reagents optimized for studies in sphingolipid metabolism, cellular signaling, and neurological disorders.

    Structurally, Non-lysosomal glucosylceramidase belongs to the glycoside hydrolase family 116 and contains a catalytic domain with a conserved nucleophilic residue required for glucosyl transfer. The enzyme is anchored to membranes through hydrophobic regions and operates in concert with lipid transporters. This antibody facilitates investigation of GBA2's role in non-lysosomal glycosphingolipid metabolism and neurological function.

    UniProt

    Q9HCG7
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