| 產(chǎn)品編號(hào) | bs-12706R-BF488 |
| 英文名稱1 | Rabbit Anti-B4GALNT1/GM2 synthase/BF488 Conjugated antibody |
| 中文名稱 | BF488標(biāo)記的β1,4-N乙酰半乳糖氨基轉(zhuǎn)移酶1抗體 |
| 別 名 | (N acetylneuraminyl) galactosylglucosylceramide; (N-acetylneuraminyl)-galactosylglucosylceramide; 4 N-acetylgalactosaminyltransferase 1; 4933429D13Rik; B4GALNT1; B4GN1_HUMAN; Beta 1 4 N acetyl galactosaminyl transferase 1; Beta 1 4 N acetylgalactosaminyltransferase 1; Beta-1; BETA-1,4-N-ACETYLGALACTOSAMINYLTRANSFERASE 1; BETA-1,4-N-ACETYLGALACTOSAMINYLTRANSFERASE; Beta1 4GalNAc T; Gal-NAc-T; GALGT; Galgt1; GalNAc T; GalNAc-T; GALNACT; GD2 synthase; Ggm-2; Ggm2; GM2 synthase; GM2/GD2 synthase; SIAT 2; SIAT2; UDP Gal:betaGlcNAc beta 1 4 N acetylgalactosaminyltransferase transferase 1; UDP N acetyl alpha D galactosamine:(N acetylneuraminyl). |
| 規(guī)格價(jià)格 | 100ul/2980元 購(gòu)買(mǎi) 大包裝/詢價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 轉(zhuǎn)錄調(diào)節(jié)因子 新陳代謝 |
| 抗體來(lái)源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Mouse, (predicted: Human, Rat, Dog, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)品應(yīng)用 | ICC=1:50-200 IF=1:50-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 59kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human B4GALNT1/GM2 synthase |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 儲(chǔ) 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol |
| 保存條件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 產(chǎn)品介紹 |
background: GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. GalNAc-T is the enzyme involved in the biosynthesis of G(M2) and G(D2) glycosphingolipids. GalNAc-T catalyzes the transfer of GalNAc into G(M3) and G(D3) by a beta-1,4 linkage, resulting in the synthesis of G(M2) and G(D2), respectively. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2013] Function: Involved in the biosynthesis of gangliosides GM2, GD2 and GA2. Subunit: Homodimer; disulfide-linked. Subcellular Location: Golgi apparatus membrane; Single-pass type II membrane protein. DISEASE: Spastic paraplegia 26, autosomal recessive (SPG26) [MIM:609195]: A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body. SPG26 is a complicated form characterized by onset in the first 2 decades of life of gait abnormalities due to lower limb spasticity and muscle weakness. Some patients have upper limb involvement. Additional features include intellectual disability, peripheral neuropathy, dysarthria, cerebellar signs, extrapyramidal signs, and cortical atrophy. The disorder is slowly progressive. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the glycosyltransferase 2 family. Database links: Entrez Gene: 2583 Human Entrez Gene: 14421 Mouse Omim: 601873 Human SwissProt: Q00973 Human SwissProt: Q09200 Mouse Unigene: 159481 Human Unigene: 386762 Mouse Unigene: 10119 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
