Galactosylceramidase Polyclonal Antibody

$Lane 1: Human HUVEC cell Lysates; Lane 2: Human U-2 OScell Lysates. Probed with Galactosylceramidase polyclonal Antibody, unconjugated (bs-20477R) at 1:1000 dilution and 4\u00b0C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37\u02daC.$

Lane 1: Human HUVEC cell Lysates; Lane 2: Human U-2 OScell Lysates. Probed with Galactosylceramidase polyclonal Antibody, unconjugated (bs-20477R) at 1:1000 dilution and 4\u00b0C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37\u02daC.

Applications

IHC-P
IHC-F
IF(ICC)
IF

Reactivity

Human
Rat

Predicted Reactivity

Mouse
Dog
Cow
Sheep
Pig
Horse
Rabbit

Overview
Catalog #	bs-20477r
Product Name	Galactosylceramidase Polyclonal Antibody
Applications	IHC-P IHC-F IF(ICC) IF
Reactivity	Human, Rat
Predicted Reactivity	Mouse, Dog, Cow, Sheep, Pig, Horse, Rabbit
Specifications
Conjugation	Unconjugated
Host	Rabbit
Source	KLH conjugated synthetic peptide derived from human Galactosylceramidase
Immunogen Range	211-320/685
Clonality	Polyclonal
Isotype	IgG
Concentration	1ug/ul
Purification	Purified by Protein A.
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage Condition	Store at -20°C for 12 months.
Target
Gene ID	2581
Swiss Prot	P54803
Synonyms	Gacy; Galactocerebrosidase; Galactocerebroside beta galactosidase; Galactosylceramide beta galactosidase; galactosylceraminidase; Galc; GALCERase; Twitcher; GALC_HUMAN.
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.
Application Dilution
IHC-P
IHC-F
IF(ICC)
IF