NP_001037727.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
NCBI Official Full Name
galactocerebrosidase
NCBI Protein Information
galactocerebrosidase; GALCERase; galactocerebroside beta-galactosidase; galactosylceramide beta-galactosidase
UniProt Protein Name
Galactocerebrosidase
UniProt Synonym Protein Names
Galactocerebroside beta-galactosidase; Galactosylceramidase; Galactosylceramide beta-galactosidase
UniProt Synonym Gene Names
UniProt Entry Name
GALC_MACMU
UniProt Comments for GALC
Function: Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. 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
By similarity.
Catalytic activity: D-galactosyl-N-acylsphingosine + H2O = D-galactose + N-acylsphingosine.
Subcellular location: Lysosome
By similarity.
Involvement in disease: Note=Defects in GALC are the cause of globoid cell leukodystrophy (GLD); also known as Krabbe disease. This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin.
Sequence similarities: Belongs to the glycosyl hydrolase 59 family.
Caution: It is uncertain whether Met-1 or Met-17 is the initiator.
Sequence caution: The sequence AAB58575.1 differs from that shown. Reason: Erroneous initiation.
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Pathways associated with GALC recombinant protein
Diseases associated with GALC recombinant protein
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