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B4GALNT1/GM2 synthase Rabbit pAb (bs-12706R)  
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產(chǎn)品編號 bs-12706R
英文名稱 B4GALNT1/GM2 synthase Rabbit pAb
中文名稱 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).  
研究領域 細胞生物  神經(jīng)生物學  信號轉(zhuǎn)導  轉(zhuǎn)錄調(diào)節(jié)因子  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,Mouse,Rat (predicted: Rabbit,Sheep,Cow,Dog,Horse)
產(chǎn)品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 59 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 細胞外基質(zhì) 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human B4GALNT1/GM2 synthase: 451-533/533 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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



產(chǎn)品圖片
Sample: Stomach (Mouse) Lysate at 40 ug Primary: Anti- B4GALNT1/GM2 synthase (bs-12706R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 59 kD Observed band size: 59 kD
Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti- B4GALNT1/GM2 synthase (bs-12706R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 59 kD Observed band size: 53 kD
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; An
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; An
Paraformaldehyde-fixed, paraffin embedded (human brain glioma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30
Paraformaldehyde-fixed, paraffin embedded (rat cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min;
Paraformaldehyde-fixed, paraffin embedded (Mouse cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30mi
Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Anti
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