產(chǎn)品詳情
簡單介紹：
Anti-HEXA抗體產(chǎn)品質(zhì)量穩(wěn)定，實驗效果明顯，貨期快，價格優(yōu)惠，歡迎垂詢訂購！我公司長期供應(yīng)**組化抗體、WB抗體、**組化試劑盒和抗體試驗所需全部相關(guān)試劑、熒光標(biāo)記抗體、單克隆抗體、多克隆抗體、各種標(biāo)記的二抗IgG/IgM/IgD/IgA等科研實驗抗體。Anti-HEXA抗體用于**組化實驗,WB實驗,相應(yīng)的標(biāo)記抗體有HRP標(biāo)記抗體,FITC標(biāo)記,BIO等。
詳情介紹：
Rabbit Anti-HEXA
Cat. Number:
Anti-HEXA抗體KL-11726R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one ? chain, one ∫-A chain and one ∫-B chain and is found in the lysosomes of cells. HEXA, along with the cofactor CM2 activator protein, catalyzes the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines in the brain and other tissues. A mutation in the ? subunit of hexosaminidase is the cause of Anti-HEXA抗體Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.
Also known as:
Beta hexosaminidase alpha chain Anti-HEXA抗體precursor; Beta hexosaminidase subunit alpha; Beta N acetylhexosaminidase; Beta N acetylhexosaminidase subunit alpha; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexa; HEXA_HUMAN; Hexosaminidase A (alpha polypeptide); Hexosaminidase A alpha polypeptide; Hexosaminidase A; Hexosaminidase subunit A; MGC99608; N acetyl beta glucosaminidase; N acetyl beta glucosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha; TSD.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, .
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Immunogen: KLH conjugated synthetic peptide derived from human Beta hex.
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Predicted Molecular Weight: 51kDa.Anti-HEXA抗體
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
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
Not yet tested in other applications. Anti-HEXA抗體
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-HEXA抗體
Optimal working dilutions must be determined by the end user.
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