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Protein

Galactocerebrosidase

Gene

GALC

Organism
Macaca mulatta (Rhesus macaque)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at protein leveli

Functioni

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.1 Publication

Catalytic activityi

D-galactosyl-N-acylsphingosine + H2O = D-galactose + N-acylsphingosine.1 Publication

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei109 – 1091SubstrateBy similarity
Binding sitei151 – 1511SubstrateBy similarity
Binding sitei197 – 1971SubstrateBy similarity
Active sitei198 – 1981Proton donor/acceptorBy similarity
Active sitei274 – 2741NucleophileBy similarity
Binding sitei396 – 3961SubstrateBy similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Glycosidase, Hydrolase

Keywords - Biological processi

Lipid degradation, Lipid metabolism, Sphingolipid metabolism

Enzyme and pathway databases

BRENDAi3.2.1.46. 3126.

Protein family/group databases

CAZyiGH59. Glycoside Hydrolase Family 59.

Names & Taxonomyi

Protein namesi
Recommended name:
Galactocerebrosidase (EC:3.2.1.46)
Short name:
GALCERase
Alternative name(s):
Galactocerebroside beta-galactosidase
Galactosylceramidase
Galactosylceramide beta-galactosidase
Gene namesi
Name:GALC
OrganismiMacaca mulatta (Rhesus macaque)
Taxonomic identifieri9544 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniCercopithecidaeCercopithecinaeMacaca
Proteomesi
  • UP000006718 Componenti: Chromosome 7

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Lysosome

Pathology & Biotechi

Involvement in diseasei

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.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 4242By similarityAdd
BLAST
Chaini43 – 685643GalactocerebrosidasePRO_0000012231Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Glycosylationi143 – 1431N-linked (GlcNAc...)Sequence analysis
Disulfide bondi287 ↔ 394By similarity
Glycosylationi379 – 3791N-linked (GlcNAc...)Sequence analysis
Glycosylationi403 – 4031N-linked (GlcNAc...)Sequence analysis
Glycosylationi451 – 4511N-linked (GlcNAc...)Sequence analysis
Glycosylationi556 – 5561N-linked (GlcNAc...)Sequence analysis
Glycosylationi559 – 5591N-linked (GlcNAc...)Sequence analysis
Glycosylationi602 – 6021N-linked (GlcNAc...)Sequence analysis

Keywords - PTMi

Disulfide bond, Glycoprotein

Expressioni

Gene expression databases

BgeeiENSMMUG00000017261.

Interactioni

Protein-protein interaction databases

STRINGi9544.ENSMMUP00000022713.

Structurei

3D structure databases

ProteinModelPortaliO02791.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the glycosyl hydrolase 59 family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

eggNOGiENOG410IITE. Eukaryota.
ENOG410XTIS. LUCA.
GeneTreeiENSGT00390000003303.
HOGENOMiHOG000068033.
HOVERGENiHBG005800.
InParanoidiO02791.
KOiK01202.
OMAiKHSKCIR.
OrthoDBiEOG091G04GY.
TreeFamiTF312985.

Family and domain databases

Gene3Di3.20.20.80. 1 hit.
InterProiIPR001286. Glyco_hydro_59.
IPR013781. Glyco_hydro_catalytic_dom.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]
PANTHERiPTHR15172. PTHR15172. 1 hit.
PfamiPF02057. Glyco_hydro_59. 1 hit.
[Graphical view]
PRINTSiPR00850. GLHYDRLASE59.
SUPFAMiSSF51445. SSF51445. 1 hit.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

O02791-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAEWLLSASR QRRVKAMTAA AGSAGRAAVP FLLCALLAPG GAYVLDDSDG
60 70 80 90 100
LGREFDGIGA VSGGGATSRL LVNYPEPYRS QILDYLFKPN FGASLHILKV
110 120 130 140 150
EIGGDGQTTD GTEPSHMHYA LDENYFRGYE WWLMKEAKKR NPNITLIGLP
160 170 180 190 200
WSFPGWLGKG FDWPYVNLQL TAYYVVTWIV GAKRYHDLDI DYIGIWNERS
210 220 230 240 250
YNANYIKILR KMLNSQGLQR VKIIASDNLW ESISAAMLLD AELFKVVDVI
260 270 280 290 300
GAHYPGTHSV KDARLTGKKL WSSEDFSTLN SDTGAGCWGR ILNQNYVNGY
310 320 330 340 350
MTSTIAWNLV ASYYEQLPYG RCGLMTAQEP WSGHYVVESP VWVSAHTTQF
360 370 380 390 400
TQPGWYYLKT VGHLEKGGSY VALTDGLGNL TIIIETMSHK HSKCIRPFLP
410 420 430 440 450
YFNVSQQFAT FVLKGSFSEI PELQVWYTKL GKTSERFLFK QLDSLWLLDS
460 470 480 490 500
NGSFTLKLQE DELFTLTTLT TGRKGSYLPP PKSQRFPSTY KDDFNVDYPF
510 520 530 540 550
FSEAPNFADQ TGVFEYFTNM EDPGEHHFTL RQVLNQRPIT WAADASNTIS
560 570 580 590 600
IIGDYNWTNL TIKCDVYIET PDTGGVFIAG RVNKGGILIR SARGIFFWIF
610 620 630 640 650
ANGSYRVTGD LAGWIIYALG HVEVTAKTWY TLTLTIKGRF ASGMLNDKSL
660 670 680
WTDIPVNFPK NGWAAIGTHS FEFAQFDNFH VEATR
Length:685
Mass (Da):77,090
Last modified:May 5, 2009 - v2
Checksum:i6087E861AC784F13
GO

Sequence cautioni

The sequence AAB58575 differs from that shown. Reason: Erroneous initiation. Curated

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U87477
, U87462, U87463, U87464, U87465, U87466, U87467, U87468, U87469, U87470, U87471, U87472, U87473, U87474, U87475, U87476 Genomic DNA. Translation: AAB58575.1. Different initiation.
U87628 mRNA. Translation: AAB58576.1.
AB172678 mRNA. No translation available.
RefSeqiNP_001037727.1. NM_001044262.2.
UniGeneiMmu.3412.

Genome annotation databases

EnsembliENSMMUT00000024269; ENSMMUP00000022713; ENSMMUG00000017261.
GeneIDi693322.
KEGGimcc:693322.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U87477
, U87462, U87463, U87464, U87465, U87466, U87467, U87468, U87469, U87470, U87471, U87472, U87473, U87474, U87475, U87476 Genomic DNA. Translation: AAB58575.1. Different initiation.
U87628 mRNA. Translation: AAB58576.1.
AB172678 mRNA. No translation available.
RefSeqiNP_001037727.1. NM_001044262.2.
UniGeneiMmu.3412.

3D structure databases

ProteinModelPortaliO02791.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9544.ENSMMUP00000022713.

Protein family/group databases

CAZyiGH59. Glycoside Hydrolase Family 59.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMMUT00000024269; ENSMMUP00000022713; ENSMMUG00000017261.
GeneIDi693322.
KEGGimcc:693322.

Organism-specific databases

CTDi2581.

Phylogenomic databases

eggNOGiENOG410IITE. Eukaryota.
ENOG410XTIS. LUCA.
GeneTreeiENSGT00390000003303.
HOGENOMiHOG000068033.
HOVERGENiHBG005800.
InParanoidiO02791.
KOiK01202.
OMAiKHSKCIR.
OrthoDBiEOG091G04GY.
TreeFamiTF312985.

Enzyme and pathway databases

BRENDAi3.2.1.46. 3126.

Gene expression databases

BgeeiENSMMUG00000017261.

Family and domain databases

Gene3Di3.20.20.80. 1 hit.
InterProiIPR001286. Glyco_hydro_59.
IPR013781. Glyco_hydro_catalytic_dom.
IPR017853. Glycoside_hydrolase_SF.
[Graphical view]
PANTHERiPTHR15172. PTHR15172. 1 hit.
PfamiPF02057. Glyco_hydro_59. 1 hit.
[Graphical view]
PRINTSiPR00850. GLHYDRLASE59.
SUPFAMiSSF51445. SSF51445. 1 hit.
ProtoNetiSearch...

Entry informationi

Entry nameiGALC_MACMU
AccessioniPrimary (citable) accession number: O02791
Entry historyi
Integrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: May 5, 2009
Last modified: September 7, 2016
This is version 90 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Caution

It is uncertain whether Met-1 or Met-17 is the initiator.Curated

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Glycosyl hydrolases
    Classification of glycosyl hydrolase families and list of entries
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.