產(chǎn)品詳情
簡(jiǎn)單介紹：
Anti-Galactocerebroside抗體產(chǎn)品質(zhì)量穩(wěn)定，實(shí)驗(yàn)效果明顯，貨期快，價(jià)格優(yōu)惠，歡迎垂詢(xún)訂購(gòu)！我公司長(zhǎng)期供應(yīng)**組化抗體、WB抗體、**組化試劑盒和抗體試驗(yàn)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(shí)驗(yàn)抗體。Anti-Galactocerebroside抗體用于**組化實(shí)驗(yàn),WB實(shí)驗(yàn),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-Galactocerebroside
Cat. Number:
Anti-Galactocerebroside抗體KL-4691R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Galactosylceramidase hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. It is an enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. It shows highest level of activity in testes compared to brain, kidney, Anti-Galactocerebroside抗體placenta and liver. It can also be found in urine. Defects in Galactosylceramidase are the cause of globoid cell leukodystrophy (GLD); also known as Krabbe disease. This autosomal recessive disorder results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Clinically, the most frequent form is the infantile form. Most patients (90%) present before six months of age with irritability, spasticity, arrest of motor and mental development, and bouts of temperature elevation without infection. This is followed by myoclonic jerks of arms and legs, oposthotonus, hypertonic fits, and mental regression, which progresses to a severe decerebrate condition with no voluntary movements and death from respiratory infections or cerebral hyperpyrexia before 2 years of age. However, a significant number of cases with later onset, presenting with unexplained blindness, weakness and/or progressive motor, and sensory neuropathy that can progress to severe mental incapacity and death, have been identified.
Also known as:
Gacy; Galc; Galactocerebrosidase; Anti-Galactocerebroside抗體Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; Galc; GALCERase; Twitcher.
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, Guinea Pig, .
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Immunogen: KLH conjugated synthetic peptide derived from human Galc.
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Predicted Molecular Weight: 71kDa.Anti-Galactocerebroside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 IF=1:100-500
Not yet tested in other applications. Anti-Galactocerebroside抗體
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-Galactocerebroside抗體
Optimal working dilutions must be determined by the end user.
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