| 10651 | Glb1 | galactosidase, beta 1 | This gene encodes a preproprotein that is proteolytically cleaved to yield a signal peptide and a proproptein that is subsequently processed to generate the active mature peptide. The encoded protein is a lysosomal enzyme that catalyzes the hydrolysis of terminal beta-D-galactose residues in various substrates like lactose, ganglioside GM1 and other glycoproteins. Mutations in the human gene are associated with GM1-gangliosidosis and Morquio B syndrome. Disruption of the mouse gene mirrors the symptoms of human gangliosidosis. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2013] | 9 | 114230146 | 114303447 | Mouse | 367 | symbol , PhenoGen | gene, protein-coding, REVIEWED [RefSeq] |
| 1347574 | GLB1 | galactosidase beta 1 | This gene encodes a member of the glycosyl hydrolase 35 family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate the mature lysosomal enzyme. This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. Mutations in this gene may result in GM1-gangliosidosis and Morquio B syndrome. [provided by RefSeq, Nov 2015] | 3 | 32961108 | 33097146 | Human | 931 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, REVIEWED [RefSeq] |
| 1597145 | Glb1 | galactosidase, beta 1 | ENCODES a protein that exhibits beta-galactosidase activity; galactoside binding; hydrolase activity (ortholog); INVOLVED IN galactose catabolic process; response to cortisone; response to Thyroglobulin triiodothyronine; PARTICIPATES IN Fabry disease pathway; galactose metabolic pathway; galactosemi a pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (ortholog); extracellular space (ortholog); lysosome (ortholog); INTERACTS WITH (+)-schisandrin B; 2,3,7,8-tetrachlorodibenzodioxine; 2,4-dinitrotoluene | 8 | 122963718 | 123036326 | Rat | 322 | symbol , PhenoGen , old_gene_symbol | gene, protein-coding, PROVISIONAL [RefSeq] |
| 8953752 | Glb1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); hydrolase activity (ortholog); INVOLVED IN carbohydrate metabolic process (ortholog); galactose catabolic process (ortholog); ganglioside catabolic process (ortholog); PARTICIPATES IN Fabry diseas e pathway; galactose metabolic pathway; galactosemia pathway; ASSOCIATED WITH Otitis Media with Effusion; atopic dermatitis (ortholog); bone development disease (ortholog); FOUND IN cytoplasm (ortholog); extracellular space (ortholog); lysosome (ortholog) | NW_004955421 | 230957 | 311504 | Chinchilla | 62 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 11755893 | GLB1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); INVOLVED IN galactose catabolic process (ortholog); response to cortisone (ortholog); response to Thyroglobulin triiodothyronine (ortholog); PARTICIPATES IN Fabry disease pathway; galactose metabo lic pathway; galactosemia pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (inferred); extracellular region (inferred); extracellular space (inferred) | 3 | 32892039 | 33031959 | Bonobo | 68 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12221620 | GLB1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); INVOLVED IN galactose catabolic process (ortholog); response to cortisone (ortholog); response to Thyroglobulin triiodothyronine (ortholog); PARTICIPATES IN Fabry disease pathway; galactose metabo lic pathway; galactosemia pathway; ASSOCIATED WITH GM1 gangliosidosis; atopic dermatitis (ortholog); bone development disease (ortholog); FOUND IN cytoplasm (inferred); extracellular region (inferred); extracellular space (inferred) | 23 | 3721768 | 3814209 | Dog | 70 | symbol | gene, protein-coding, PROVISIONAL [RefSeq] |
| 12533144 | Glb1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); INVOLVED IN galactose catabolic process (ortholog); response to cortisone (ortholog); response to Thyroglobulin triiodothyronine (ortholog); PARTICIPATES IN Fabry disease pathway; galactose metabo lic pathway; galactosemia pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (inferred); extracellular region (inferred); extracellular space (inferred) | NW_004936473 | 23348431 | 23440271 | Squirrel | 68 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14270675 | GLB1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); INVOLVED IN galactose catabolic process (ortholog); response to cortisone (ortholog); response to Thyroglobulin triiodothyronine (ortholog); PARTICIPATES IN Fabry disease pathway; galactose metabo lic pathway; galactosemia pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (inferred); extracellular region (inferred); extracellular space (inferred); INTERACTS WITH deoxynivalenol | | | | Pig | 72 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18738031 | GLB1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); INVOLVED IN galactose catabolic process (ortholog); response to cortisone (ortholog); response to Thyroglobulin triiodothyronine (ortholog); PARTICIPATES IN Fabry disease pathway; galactose metabo lic pathway; galactosemia pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (inferred); extracellular region (inferred); extracellular space (inferred) | | | | Green Monkey | 68 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18901819 | Glb1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); protein homodimerization activity (ortholog); INVOLVED IN galactose catabolic process (ortholog); glycoprotein catabolic process (ortholog); response to cortisone (ortholog); PARTICIPATES IN Fabry disease pathway; galactose metabolic pathway; galactosemia pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (ortholog); extracellular space (ortholog); lysosome (ortholog) | | | | Naked Mole-Rat | 64 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155235696 | glb1 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 625855616 | Glb1 | galactosidase beta 1 | ENCODES a protein that exhibits beta-galactosidase activity (ortholog); galactoside binding (ortholog); hydrolase activity (ortholog); INVOLVED IN carbohydrate metabolic process (ortholog); galactose catabolic process (ortholog); ganglioside catabolic process (ortholog); PARTICIPATES IN Fabry diseas e pathway; galactose metabolic pathway; galactosemia pathway; ASSOCIATED WITH atopic dermatitis (ortholog); bone development disease (ortholog); contact dermatitis (ortholog); FOUND IN cytoplasm (ortholog); extracellular space (ortholog); lysosome (ortholog) | | | | Black Rat | 60 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155249629 | glb1.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 1309474 | Glb1l | galactosidase, beta 1-like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog); INTERACTS WITH 2,3,7,8-tet rachlorodibenzodioxine; 3-chloropropane-1,2-diol; 6-propyl-2-thiouracil | 9 | 84141993 | 84154176 | Rat | 55 | symbol , PhenoGen | gene, protein-coding, PROVISIONAL [RefSeq] |
| 1319832 | GLB1L | galactosidase beta 1 like | Predicted to enable beta-galactosidase activity. Predicted to be involved in galactose catabolic process. Predicted to be located in extracellular region. Predicted to be active in vacuole. [provided by Alliance of Genome Resources, Jul 2025] | 2 | 219236598 | 219245444 | Human | 55 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, VALIDATED [RefSeq] |
| 1611811 | Glb1l | galactosidase, beta 1-like | Predicted to enable beta-galactosidase activity. Predicted to be involved in galactose catabolic process. Located in extracellular space. Orthologous to human GLB1L (galactosidase beta 1 like). [provided by Alliance of Genome Resources, Jul 2025] | 1 | 75174178 | 75187460 | Mouse | 57 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 8731598 | Glb1l | galactosidase beta 1 like | ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog) | NW_004955453 | 14185582 | 14196779 | Chinchilla | 1 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 11689573 | GLB1L | galactosidase beta 1 like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog) | 2B | 106494214 | 106503001 | Bonobo | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12188393 | GLB1L | galactosidase beta 1 like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog); INTERACTS WITH bisphenol A | 37 | 25783837 | 25793968 | Dog | 10 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12631665 | Glb1l | galactosidase beta 1 like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog) | NW_004936569 | 1653482 | 1663978 | Squirrel | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14104240 | GLB1L | galactosidase beta 1 like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog) | | | | Pig | 9 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18630118 | GLB1L | galactosidase beta 1 like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog) | | | | Green Monkey | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18903193 | Glb1l | galactosidase beta 1 like | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); ASSOCIATED WITH cerebrotendinous xanthomatosis (ortholog) | | | | Naked Mole-Rat | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155246410 | glb1l | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 625922291 | Glb1l | galactosidase beta 1 like | | | | | Black Rat | | symbol | gene, protein-coding, MODEL [RefSeq] |
| 1564436 | Glb1l2 | galactosidase, beta 1-like 2 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); INTERACTS WITH 17beta-estradiol; 2,3,7,8-tetrachlorodibenzodioxine; aflatoxin B1 | 8 | 33391201 | 33442587 | Rat | 63 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 1606483 | GLB1L2 | galactosidase beta 1 like 2 | Predicted to enable beta-galactosidase activity. Predicted to be involved in galactose catabolic process. Predicted to be located in extracellular region. Predicted to be active in vacuole. [provided by Alliance of Genome Resources, Jul 2025] | 11 | 134331988 | 134376324 | Human | 63 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, VALIDATED [RefSeq] |
| 1621283 | Glb1l2 | galactosidase, beta 1-like 2 | Predicted to enable beta-galactosidase activity. Predicted to be involved in galactose catabolic process. Predicted to be located in extracellular region. Predicted to be active in vacuole. Is expressed in foregut-midgut junction; molar; nose; urinary system; and vestibulo-cochlear ganglion. Ortholo gous to human GLB1L2 (galactosidase beta 1 like 2). [provided by Alliance of Genome Resources, Jul 2025] | 9 | 26674338 | 26717769 | Mouse | 67 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 11955081 | GLB1L2 | galactosidase beta 1 like 2 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | 11 | 129184833 | 129233585 | Bonobo | 7 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14254996 | GLB1L2 | galactosidase beta 1 like 2 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | | | | Pig | 10 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 18740963 | GLB1L2 | galactosidase beta 1 like 2 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | | | | Green Monkey | 7 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 284476649 | glb1l2 | | | | | | Tropical Clawed Frog | | symbol | gene, null |
| 1556861 | Glb1l3 | galactosidase, beta 1 like 3 | Predicted to enable beta-galactosidase activity. Predicted to be involved in galactose catabolic process. Predicted to be active in vacuole. Orthologous to human GLB1L3 (galactosidase beta 1 like 3). [provided by Alliance of Genome Resources, Jul 2025] | 9 | 26729249 | 26773394 | Mouse | 47 | symbol , PhenoGen , description | gene, protein-coding, VALIDATED [RefSeq] |
| 1561462 | Glb1l3 | galactosidase, beta 1-like 3 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); INTERACTS WITH 17beta-estradiol; 17beta-estradiol 3-benzoate; 3,3',4,4',5-pentachloro biphenyl | 8 | 33454914 | 33496641 | Rat | 46 | symbol , PhenoGen | gene, protein-coding, VALIDATED [RefSeq] |
| 1603928 | GLB1L3 | galactosidase beta 1 like 3 | Predicted to enable beta-galactosidase activity. Predicted to be involved in galactose catabolic process. Predicted to be active in vacuole. [provided by Alliance of Genome Resources, Jul 2025] | 11 | 134275767 | 134325304 | Human | 46 | symbol , COSMIC , Human Proteome Map | gene, protein-coding, VALIDATED [RefSeq] |
| 11984146 | GLB1L3 | galactosidase beta 1 like 3 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | 11 | 129126472 | 129172535 | Bonobo | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 12105373 | GLB1L3 | galactosidase beta 1 like 3 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | 5 | 805531 | 846125 | Dog | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 14106376 | GLB1L3 | galactosidase beta 1 like 3 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | | | | Pig | 9 | symbol , ensembl_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 18606312 | GLB1L3 | galactosidase beta 1 like 3 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | | | | Green Monkey | 6 | symbol | gene, protein-coding, MODEL [RefSeq] |
| 626007765 | Glb1l3 | galactosidase beta 1 like 3 | | | | | Black Rat | | symbol | gene, protein-coding, MODEL [RefSeq] |
| 155246411 | glb1l.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 287655535 | glb1l2.L | | | | | | African Clawed Frog | | symbol | gene, null |
| 285507780 | glb1l2.S | | | | | | African Clawed Frog | | symbol | gene, null |
| 1359233 | Bin2a | beta-galactosidase-like protein | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred); INTERACTS WITH bisphenol A; fenvalerate | 8 | 33524302 | 33562904 | Rat | 8 | old_gene_symbol | gene, protein-coding, VALIDATED [RefSeq] |
| 18925203 | LOC101710289 | beta-galactosidase-1-like protein 2 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | | | | Naked Mole-Rat | 6 | old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 12576697 | LOC101976490 | beta-galactosidase-1-like protein 2 | | NW_004936684 | 1277765 | 1304851 | Squirrel | 1 | old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 12479637 | LOC101977044 | beta-galactosidase-1-like protein 3 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | NW_004936684 | 1414926 | 1447960 | Squirrel | 6 | old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 8909440 | LOC102004261 | beta-galactosidase-1-like protein 2 | | NW_004955412 | 35259632 | 35292661 | Chinchilla | | ensembl_gene_symbol , old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 9067337 | LOC102006113 | beta-galactosidase-1-like protein 3 | | NW_004955412 | 35224061 | 35251279 | Chinchilla | | old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 12284371 | LOC606786 | beta-galactosidase-1-like protein 2 | ENCODES a protein that exhibits beta-galactosidase activity (inferred); hydrolase activity (inferred); hydrolase activity, acting on glycosyl bonds (inferred); INVOLVED IN carbohydrate metabolic process (inferred) | 5 | 752134 | 791824 | Dog | 10 | ensembl_gene_symbol , old_gene_symbol | gene, protein-coding, MODEL [RefSeq] |
| 16567850 | AC003001.2 | galactosidase, beta 1-like (GLB1L) pseudogene | | | | | Human | | name | gene, processed_pseudogene |