Immunogen
Synthesized peptide derived from human GALC AA range: 481-531
Specificity
This antibody detects endogenous levels of GALC at Human/Mouse
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1：500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
This gene encodes a lysosomal protein which hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Mutations in this gene have been associated with Krabbe disease, also known as globoid cell leukodystrophy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Jul 2008],
Function
catalytic activity:D-galactosyl-N-acylsphingosine + H(2)O = D-galactose + N-acylsphingosine.,caution:It is uncertain whether Met-1 or Met-17 is the initiator.,disease:Defects in GALC are the cause of leukodystrophy globoid cell (GLD) [MIM:245200]; 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 cereb
Fields
>>Sphingolipid metabolism;>>Metabolic pathways;>>Lysosome
Human protein sequence Database
P54803
Mouse protein sequence database
P54818
Rat protein sequence database
Cellular localization
Lysosome.
Tissue expression
Detected in urine. Detected in testis, brain and placenta (at protein level). Detected in kidney and liver.
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