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Rabbit Anti-B4GALNT1/GM2 synthase/Cy5 Conjugated antibody (bs-12706R-Cy5) ~~~促销,代码KX240301~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-12706R-Cy5
英文名称 Anti-B4GALNT1/GM2 synthase/Cy5
中文名称 Cy5标记的β1,4-N乙酰半乳糖氨基转移酶1抗体
别    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).  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  转录调节因子  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,  (predicted: Human, Rat, Dog, Cow, Horse, Rabbit, Sheep, )
产品应用 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    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human B4GALNT1/GM2 synthase
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
产品介绍 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

Entrez Gene: 64828 Rat

Omim: 601873 Human

SwissProt: Q00973 Human

SwissProt: Q09200 Mouse

SwissProt: Q10468 Rat

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.
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