β1,4-N乙酰半乳糖氨基转移酶1抗体
规格：1mg/1ml
英文名： B4GALNT1/GM2 synthase
别名： (N acetylneuraminyl) galactosylglucosylceramide; (N-acetylneuraminyl)-galactosylglucosylceramide; 4 N-acetylgalactosaminyltransferase 1; 4933429D13Rik; B4GALNT1; B4GN1_HUMAN; Beta 1 4 N acetyl galacto
分子量： 59kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human B4GALNT1
交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆 细胞膜 细胞外基质
β1,4-N乙酰半乳糖氨基转移酶1抗体产品介绍：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, β1,4-N乙酰半乳糖氨基转移酶1抗体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 spasticityβ1,4-N乙酰半乳糖氨基转移酶1抗体 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. 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.
β1,4-N乙酰半乳糖氨基转移酶1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 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.
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
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid