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Protein

Galactocerebrosidase

Gene

Galc

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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

Caution

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

Catalytic activityi

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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei109Substrate1
Binding sitei151Substrate1
Binding sitei197Substrate1
Active sitei198Proton donor/acceptor1 Publication1
Active sitei274Nucleophile1 Publication1
Binding sitei396Substrate1

GO - Molecular functioni

  • galactosylceramidase activity Source: UniProtKB

GO - Biological processi

  • galactosylceramide catabolic process Source: UniProtKB

Keywordsi

Molecular functionGlycosidase, Hydrolase
Biological processLipid degradation, Lipid metabolism, Sphingolipid metabolism

Enzyme and pathway databases

BRENDAi3.2.1.46 3474
ReactomeiR-MMU-1660662 Glycosphingolipid metabolism

Protein family/group databases

CAZyiGH59 Glycoside Hydrolase Family 59

Chemistry databases

SwissLipidsiSLP:000001418

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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 12

Organism-specific databases

MGIiMGI:95636 Galc

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Lysosome

Pathology & Biotechi

Involvement in diseasei

Defects in Galc are the cause of the 'twitcher' phenotype; an autosomal recessive leukodystrophy similar to the human disease (Krabbe disease). This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin.

Chemistry databases

ChEMBLiCHEMBL2218

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 42By similarityAdd BLAST42
ChainiPRO_000001223243 – 684GalactocerebrosidaseAdd BLAST642

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi287 ↔ 3941 Publication
Glycosylationi300N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi379N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi403N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi558N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi601N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi645N-linked (GlcNAc...) asparagineSequence analysis1

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP54818
PaxDbiP54818
PeptideAtlasiP54818
PRIDEiP54818

PTM databases

iPTMnetiP54818
PhosphoSitePlusiP54818

Expressioni

Tissue specificityi

Detected in brain and kidney.1 Publication

Gene expression databases

BgeeiENSMUSG00000021003 Expressed in 242 organ(s), highest expression level in stroma of bone marrow
CleanExiMM_GALC
ExpressionAtlasiP54818 baseline and differential
GenevisibleiP54818 MM

Interactioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000021390

Structurei

Secondary structure

1684
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP54818
SMRiP54818
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
InParanoidiP54818
KOiK01202
OMAiFWIFANG
OrthoDBiEOG091G04GY
PhylomeDBiP54818
TreeFamiTF312985

Family and domain databases

Gene3Di3.20.20.70, 1 hit
InterProiView protein in InterPro
IPR013785 Aldolase_TIM
IPR001286 Glyco_hydro_59
IPR035394 Glyco_hydro_59_dom
IPR017853 Glycoside_hydrolase_SF
PANTHERiPTHR15172 PTHR15172, 1 hit
PfamiView protein in Pfam
PF02057 Glyco_hydro_59, 1 hit
PF17387 Glyco_hydro_59M, 1 hit
PRINTSiPR00850 GLHYDRLASE59
SUPFAMiSSF51445 SSF51445, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

P54818-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MANSQPKASQ QRQAKVMTAA AGSASRVAVP LLLCALLVPG GAYVLDDSDG
60 70 80 90 100
LGREFDGIGA VSGGGATSRL LVNYPEPYRS EILDYLFKPN FGASLHILKV
110 120 130 140 150
EIGGDGQTTD GTEPSHMHYE LDENYFRGYE WWLMKEAKKR NPDIILMGLP
160 170 180 190 200
WSFPGWLGKG FSWPYVNLQL TAYYVVRWIL GAKHYHDLDI DYIGIWNERP
210 220 230 240 250
FDANYIKELR KMLDYQGLQR VRIIASDNLW EPISSSLLLD QELWKVVDVI
260 270 280 290 300
GAHYPGTYTV WNAKMSGKKL WSSEDFSTIN SNVGAGCWSR ILNQNYINGN
310 320 330 340 350
MTSTIAWNLV ASYYEELPYG RSGLMTAQEP WSGHYVVASP IWVSAHTTQF
360 370 380 390 400
TQPGWYYLKT VGHLEKGGSY VALTDGLGNL TIIIETMSHQ HSMCIRPYLP
410 420 430 440 450
YYNVSHQLAT FTLKGSLREI QELQVWYTKL GTPQQRLHFK QLDTLWLLDG
460 470 480 490 500
SGSFTLELEE DEIFTLTTLT TGRKGSYPPP PSSKPFPTNY KDDFNVEYPL
510 520 530 540 550
FSEAPNFADQ TGVFEYYMNN EDREHRFTLR QVLNQRPITW AADASSTISV
560 570 580 590 600
IGDHHWTNMT VQCDVYIETP RSGGVFIAGR VNKGGILIRS ATGVFFWIFA
610 620 630 640 650
NGSYRVTADL GGWITYASGH ADVTAKRWYT LTLGIKGYFA FGMLNGTILW
660 670 680
KNVRVKYPGH GWAAIGTHTF EFAQFDNFRV EAAR
Length:684
Mass (Da):77,256
Last modified:May 5, 2009 - v2
Checksum:iDD4BD45AE898C524
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A1Y7VM63A0A1Y7VM63_MOUSE
Galactocerebrosidase
Galc
491Annotation score:

Sequence cautioni

The sequence AAB71823 differs from that shown. Reason: Erroneous initiation.Curated
The sequence AAH86671 differs from that shown. Reason: Erroneous initiation.Curated
The sequence BAA07560 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti376G → A in AAB71823 (Ref. 2) Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D38557 mRNA Translation: BAA07560.1 Different initiation.
AF003886
, AF003870, AF003871, AF003872, AF003873, AF003874, AF003875, AF003876, AF003877, AF003878, AF003879, AF003880, AF003881, AF003882, AF003883, AF003884, AF003885 Genomic DNA Translation: AAB71823.1 Different initiation.
AK154760 mRNA Translation: BAE32809.1
CH466549 Genomic DNA Translation: EDL18937.1
BC086671 mRNA Translation: AAH86671.1 Different initiation.
CCDSiCCDS36517.1
RefSeqiNP_032105.2, NM_008079.4
UniGeneiMm.5120

Genome annotation databases

EnsembliENSMUST00000021390; ENSMUSP00000021390; ENSMUSG00000021003
GeneIDi14420
KEGGimmu:14420
UCSCiuc007olf.2 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
D38557 mRNA Translation: BAA07560.1 Different initiation.
AF003886
, AF003870, AF003871, AF003872, AF003873, AF003874, AF003875, AF003876, AF003877, AF003878, AF003879, AF003880, AF003881, AF003882, AF003883, AF003884, AF003885 Genomic DNA Translation: AAB71823.1 Different initiation.
AK154760 mRNA Translation: BAE32809.1
CH466549 Genomic DNA Translation: EDL18937.1
BC086671 mRNA Translation: AAH86671.1 Different initiation.
CCDSiCCDS36517.1
RefSeqiNP_032105.2, NM_008079.4
UniGeneiMm.5120

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
3ZR5X-ray2.10A40-684[»]
3ZR6X-ray2.44A40-684[»]
4CCCX-ray2.09A41-684[»]
4CCDX-ray1.97A41-684[»]
4CCEX-ray2.06A41-684[»]
4UFHX-ray2.16A41-684[»]
4UFIX-ray2.40A41-684[»]
4UFJX-ray2.20A41-684[»]
4UFKX-ray2.40A43-684[»]
4UFLX-ray2.40A43-684[»]
4UFMX-ray2.40A43-684[»]
5NXBX-ray3.60A/B41-684[»]
ProteinModelPortaliP54818
SMRiP54818
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000021390

Chemistry databases

ChEMBLiCHEMBL2218
SwissLipidsiSLP:000001418

Protein family/group databases

CAZyiGH59 Glycoside Hydrolase Family 59

PTM databases

iPTMnetiP54818
PhosphoSitePlusiP54818

Proteomic databases

MaxQBiP54818
PaxDbiP54818
PeptideAtlasiP54818
PRIDEiP54818

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000021390; ENSMUSP00000021390; ENSMUSG00000021003
GeneIDi14420
KEGGimmu:14420
UCSCiuc007olf.2 mouse

Organism-specific databases

CTDi2581
MGIiMGI:95636 Galc

Phylogenomic databases

eggNOGiENOG410IITE Eukaryota
ENOG410XTIS LUCA
GeneTreeiENSGT00390000003303
HOGENOMiHOG000068033
HOVERGENiHBG005800
InParanoidiP54818
KOiK01202
OMAiFWIFANG
OrthoDBiEOG091G04GY
PhylomeDBiP54818
TreeFamiTF312985

Enzyme and pathway databases

BRENDAi3.2.1.46 3474
ReactomeiR-MMU-1660662 Glycosphingolipid metabolism

Miscellaneous databases

ChiTaRSiGalc mouse
PROiPR:P54818
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000021003 Expressed in 242 organ(s), highest expression level in stroma of bone marrow
CleanExiMM_GALC
ExpressionAtlasiP54818 baseline and differential
GenevisibleiP54818 MM

Family and domain databases

Gene3Di3.20.20.70, 1 hit
InterProiView protein in InterPro
IPR013785 Aldolase_TIM
IPR001286 Glyco_hydro_59
IPR035394 Glyco_hydro_59_dom
IPR017853 Glycoside_hydrolase_SF
PANTHERiPTHR15172 PTHR15172, 1 hit
PfamiView protein in Pfam
PF02057 Glyco_hydro_59, 1 hit
PF17387 Glyco_hydro_59M, 1 hit
PRINTSiPR00850 GLHYDRLASE59
SUPFAMiSSF51445 SSF51445, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiGALC_MOUSE
AccessioniPrimary (citable) accession number: P54818
Secondary accession number(s): O35151, Q3U3H7
Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 1, 1996
Last sequence update: May 5, 2009
Last modified: September 12, 2018
This is version 133 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

3D-structure, 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
  3. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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