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β1,4-N乙酰半乳糖氨基转移酶1抗体
  • 产品货号:
    BN40386R
  • 中文名称:
    β1,4-N乙酰半乳糖氨基转移酶1抗体
  • 英文名称:
    Rabbit anti-B4GALNT1/GM2 synthase Polyclonal antibody
  • 货号

    产品规格

    售价

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  • BN40386R-100ul

    100ul

    ¥2360.00

    交叉反应:Human,Mouse,Rat(predicted:Dog,Cow,Horse,Rabbit,Sheep) 推荐应用:WB,IHC-P,IHC-F,ICC,IF,ELISA

  • BN40386R-200ul

    200ul

    ¥3490.00

    交叉反应:Human,Mouse,Rat(predicted:Dog,Cow,Horse,Rabbit,Sheep) 推荐应用:WB,IHC-P,IHC-F,ICC,IF,ELISA

英文名称B4GALNT1/GM2 synthase
中文名称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).  
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WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量59kDa
细胞定位细胞浆 细胞膜 细胞外基质 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human B4GALNT1/GM2 synthase:451-533/533 
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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍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.

SWISS:
Q00973

Gene ID:
2583

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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