Rabbit Anti-Galactosylceramidase Polyclonal Antibody
DATASHEET
Host:Rabbit
Target Protein:Galactosylceramidase
IR:Immunogen Range:81-180/685
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:2581
Swiss Prot:P54803
Source:KLH conjugated synthetic peptide derived from human G protein-regulated inducer of neurite outgrowth 2:81-180/685
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Background:GALC is a lysosomal enzyme that hydrolyzes galactose ester bonds in various galactolipids, including galactosylceramide, galactosylsphingosine, lactosylceramide and monogalactosyldiglyceride. Galactolipids contain glucose and/or galactose, and are found in the brain and other nerve tissue, especially the myelin sheath. Galactosylceramide is a major lipid in myelin, kidney, and epithelial cells of the small intestine and colon. Mutations in the GALC gene that compromise protein function correlate to Krabbe disease (globoid cell leukodystrophy, GLD). GLD is an autosomal recessive condition that affects approximately 1 in 150,000 infants and results in progressive destruction of the nervous system. The “twitcher” mouse is a model system for GLD; the genotype is a premature stop codon (W339X) in the galactosylceramidase (GALC) gene that abolishes enzymatic activity. Alternate transcriptional splice variants, encoding different isoforms, have been characterized.
Size:50ul
Concentration:1mg/ml
Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Chicken
Dog
Pig
Cow
Horse
Rabbit
Sheep
僅供科研使用。不能用于人和動(dòng)物治療等其它臨床診斷用！
關(guān)鍵字： Galactosylceramidase;Gacy;Galactocerebrosidase;Galactocerebroside b;galactosidase;Galc;GALCERase;GALC_HUMAN;
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