Showing Protein Lysosomal acid glucosylceramidase (BMDBP00131)
| Identification | |
|---|---|
| BMDB Protein ID | BMDBP00131 |
| Secondary Accession Numbers | None |
| Name | Lysosomal acid glucosylceramidase |
| Synonyms | Not Available |
| Gene Name | GBA |
| Protein Type | Enzyme |
| Biological Properties | |
| General Function | Involved in cation binding |
| Specific Function | Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose. Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates to the PKC-activated salvage pathway of ceramide formation. Also plays a role in cholesterol metabolism. May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol. The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction. Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide. Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol. |
| Pathways |
|
| Reactions | Not Available |
| GO Classification | Not Available |
| Cellular Location | Not Available |
| Gene Properties | |
| Chromosome Location | 3 |
| Locus | Not Available |
| SNPs | GBA |
| Gene Sequence |
>1611 bp ATGGAGCTTTCAAGTCCTTCCAGAGAGGAGTATCCTATGCCTCGGGGCAGAGTAGGAATC ATGGCTGCCAGCCTCATGGGATTACTTCTACTTCATACAGTGTCGTGGGTATCAGGTGCC CGCCCCTGCAGCCCTAAAAGCTTTGGCTACAGCTCGGTGGTGTGTGTCTGCAATGGTACA TACTGTGATTCTCTGGACCCCTTGACCCTGCCTGACCCTGGCACCTTCAGCCGCTTTGAG AGCACTCGCAGCGGGCGCCGAATGGAGCTGAGTCTGGGGACCATCCAGGCCAATCGCACA GGCACAGGGTTGCTGTTGACCTTGCAGCCAGATCAGAAATTCCAGAAAGTGAAGGGATTT GGAGGGGCCATGACGGATGCTGCTGCCCTCAACATCCTTGCCCTGTCACCTGCTGCACGG AATTTGCTACTCAAATCATACTTCTCCGAAGAAGGAATTGAATACAACATCATCCGGGTC CCCATGGCCAGCTGTGACTTCTCCATCCGCACCTATACCTATGACGACAGCCCTGATGAC TTCCAGCTGCTCAACTTCAGCCTTCCAGAGGAAGATGTCAAGCTCAAGATACCACTGATT CACCAAGCTCTGGAGTTGGCCAACCGCTCTGTCTCACTCTTCGCCAGTCCCTGGACATCA CCCACTTGGCTCAAGACTAATGGGGCTGTGAATGGGAAGGGAACACTCAAGGGTCAAGCA GGGGATCTCTACCACAAGACCTGGGCCAGATACTTTGTCAAGTTCCTGGATGCCTACGCG GAGCACAAGTTACGGTTCTGGGCAGTGACAGCCGAGAACGAGCCTACTGCAGGGCTCCTT ACCGGGTACCCCTTCCAATGCTTGGGTTTCACTCCTGAGCACCAGCGAGACTTCATCGCC CGTGACCTGGGTCCCATCCTCGCCAACAGCACACACCGCGACGTCCGACTGTTGATGCTG GATGACCAGCGCTTACTGTTGCCTCGCTGGGCCCAGGTGGTGCTGGCAGACCCAGAAGCA GCTAAGTATGTTCATGGCATTGCTGTACATTGGTACCTGGACTTCCTGGCTCCAGCAAAA GCCACCCTGGGGGAGACGCACCGCCTGTTCCCCAACACCATGCTCTTTGCCTCAGAGGCC TGTGTGGGCTCCAAGTTCTGGGAGCAGAGTGTGCGGCTGGGCTCCTGGGATCGAGGGATG CGGTACAGCCACAGTATCATCACAAACCTCCTCTACCATGTGGTCGGCTGGACCGACTGG AACCTCGCCCTAAATCCTGAAGGGGGACCCAACTGGGTGCGCAATTTTGTTGATAGCCCC ATCATTGTGGATATCGCCAAGGACACATTTTACAAGCAGCCTATGTTTTACCACCTTGGC CACTTCAGCAAGTTCATTCCTGAGGGCTCGCAGAGAGTGGGGCTGGTTGCCAGTAAGAAG AGCGACTTGGACACGGTGGCACTGTTACGTCCTGACGGCTCTGCGGTTGCGGTCGTGCTG AACCGCTCCTCTAAGGACGTGCCTCTCACCATCAAGGATCCCGCCGTGGGCTTCATGGAA ACTGTCTCACCTGGCTACTCCATTCACACCTACCTGTGGCGTCGCCAGTGA |
| Protein Properties | |
| Number of Residues | 536 |
| Molecular Weight | 59855.0 |
| Theoretical pI | 8.08 |
| Pfam Domain Function | Not Available |
| Signals |
|
| Transmembrane Regions | Not Available |
| Protein Sequence |
>Glucosylceramidase MELSSPSREEYPMPRGRVGIMAASLMGLLLLHTVSWVSGARPCSPKSFGYSSVVCVCNGT YCDSLDPLTLPDPGTFSRFESTRSGRRMELSLGTIQANRTGTGLLLTLQPDQKFQKVKGF GGAMTDAAALNILALSPAARNLLLKSYFSEEGIEYNIIRVPMASCDFSIRTYTYDDSPDD FQLLNFSLPEEDVKLKIPLIHQALELANRSVSLFASPWTSPTWLKTNGAVNGKGTLKGQA GDLYHKTWARYFVKFLDAYAEHKLRFWAVTAENEPTAGLLTGYPFQCLGFTPEHQRDFIA RDLGPILANSTHRDVRLLMLDDQRLLLPRWAQVVLADPEAAKYVHGIAVHWYLDFLAPAK ATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMRYSHSIITNLLYHVVGWTDW NLALNPEGGPNWVRNFVDSPIIVDIAKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASKK SDLDTVALLRPDGSAVAVVLNRSSKDVPLTIKDPAVGFMETVSPGYSIHTYLWRRQ |
| External Links | |
| GenBank ID Protein | AAI12824.1 |
| UniProtKB/Swiss-Prot ID | Q2KHZ8 |
| UniProtKB/Swiss-Prot Entry Name | GLCM_BOVIN |
| PDB IDs | Not Available |
| GenBank Gene ID | BC112823 |
| GeneCard ID | GBA |
| GenAtlas ID | Not Available |
| HGNC ID | Not Available |
| References | |
| General References | Not Available |