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Rabbit Anti-Galactosidase alpha  antibody (bs-7593R)  
~~~促銷(xiāo),代碼KT202502A~~~
~~~促銷(xiāo),代碼KT202502B~~~
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包裝/詢(xún)價(jià)

產(chǎn)品編號(hào) bs-7593R
英文名稱(chēng) Rabbit Anti-Galactosidase alpha  antibody
中文名稱(chēng) α-半乳糖苷酶抗體
別    名 Galactosidase alpha; Alpha D galactosidase A; Alpha D galactoside galactohydrolase; Melibiase; Alpha galactosidase A; GALA; GLA; GLA protein; AGAL_HUMAN.   
研究領(lǐng)域 心血管  細(xì)胞生物  免疫學(xué)  神經(jīng)生物學(xué)  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) Human,Rat (predicted: Mouse,Rabbit,Pig,Cow,Dog)
產(chǎn)品應(yīng)用 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 45kDa
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Galactosidase alpha: 101-200/429 
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(xiàng) 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 Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.

Subunit:
Homodimer.

Subcellular Location:
Lysosome.

DISEASE:
Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.

Similarity:
Belongs to the glycosyl hydrolase 27 family.

SWISS:
P06280

Gene ID:
2717

Database links:
Entrez Gene: 2717 Human

Entrez Gene: 11605 Mouse

Entrez Gene: 363494 Rat

Omim: 300644 Human

SwissProt: P06280 Human

SwissProt: P51569 Mouse

Unigene: 69089 Human

Unigene: 1114 Mouse

 



產(chǎn)品圖片
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; Antibody incubation with (Galactosidase alpha) Polyclonal Antibody, Unconjugated (bs-7593R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Tissue/cell: human rectal carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-Galactosidase alpha Polyclonal Antibody, Unconjugated(bs-7593R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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