ID:MOGS_HUMAN DESCRIPTION: RecName: Full=Mannosyl-oligosaccharide glucosidase; EC=3.2.1.106; AltName: Full=Processing A-glucosidase I; FUNCTION: Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. CATALYTIC ACTIVITY: Exohydrolysis of the non-reducing terminal glucose residues in the mannosyl-oligosaccharide Glc(3)Man(9)GlcNAc(2). PATHWAY: Glycan metabolism; N-glycan degradation. SUBCELLULAR LOCATION: Endoplasmic reticulum membrane; Single-pass type II membrane protein. DISEASE: Defects in MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb) [MIM:606056]; also known as glucosidase I deficiency. CDGIIb is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months. SIMILARITY: Belongs to the glycosyl hydrolase 63 family. WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/GCS1";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q13724
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006457 protein folding GO:0006487 protein N-linked glycosylation GO:0008152 metabolic process GO:0009311 oligosaccharide metabolic process
KJ897787 - Synthetic construct Homo sapiens clone ccsbBroadEn_07181 MOGS gene, encodes complete protein. LF207223 - JP 2014500723-A/14726: Polycomb-Associated Non-Coding RNAs. AK292553 - Homo sapiens cDNA FLJ78566 complete cds, highly similar to Homo sapiens glucosidase I, mRNA. X87237 - H.sapiens mRNA for processing a-glucosidase I. BC002804 - Homo sapiens glucosidase I, mRNA (cDNA clone IMAGE:3637073), partial cds. BC028337 - Homo sapiens glucosidase I, mRNA (cDNA clone MGC:34915 IMAGE:5110525), complete cds. AK292719 - Homo sapiens cDNA FLJ75504 complete cds, highly similar to Homo sapiens glucosidase I, mRNA. LF363243 - JP 2014500723-A/170746: Polycomb-Associated Non-Coding RNAs. BC117250 - Homo sapiens glucosidase I, mRNA (cDNA clone MGC:150859 IMAGE:40125801), complete cds. BC117252 - Homo sapiens glucosidase I, mRNA (cDNA clone MGC:150861 IMAGE:40125803), complete cds. BC143934 - Homo sapiens cDNA clone IMAGE:9052452, with apparent retained intron. GQ891420 - Homo sapiens clone HEL-S-141 epididymis secretory sperm binding protein mRNA, complete cds. JD421766 - Sequence 402790 from Patent EP1572962. JD241122 - Sequence 222146 from Patent EP1572962. JD173861 - Sequence 154885 from Patent EP1572962. JD472790 - Sequence 453814 from Patent EP1572962. AK313355 - Homo sapiens cDNA, FLJ93881. LF363242 - JP 2014500723-A/170745: Polycomb-Associated Non-Coding RNAs. AK307991 - Homo sapiens cDNA, FLJ97939. LF363241 - JP 2014500723-A/170744: Polycomb-Associated Non-Coding RNAs. AK304655 - Homo sapiens cDNA FLJ55624 complete cds, highly similar to Mannosyl-oligosaccharide glucosidase (EC 3.2.1.106). LF363238 - JP 2014500723-A/170741: Polycomb-Associated Non-Coding RNAs. LF363237 - JP 2014500723-A/170740: Polycomb-Associated Non-Coding RNAs. DQ600658 - Homo sapiens piRNA piR-38724, complete sequence. JD027955 - Sequence 8979 from Patent EP1572962. JD025759 - Sequence 6783 from Patent EP1572962. JD444341 - Sequence 425365 from Patent EP1572962. JD367797 - Sequence 348821 from Patent EP1572962. JD058050 - Sequence 39074 from Patent EP1572962. MA598820 - JP 2018138019-A/170746: Polycomb-Associated Non-Coding RNAs. MA598819 - JP 2018138019-A/170745: Polycomb-Associated Non-Coding RNAs. MA598818 - JP 2018138019-A/170744: Polycomb-Associated Non-Coding RNAs. MA598815 - JP 2018138019-A/170741: Polycomb-Associated Non-Coding RNAs. MA598814 - JP 2018138019-A/170740: Polycomb-Associated Non-Coding RNAs. MA442800 - JP 2018138019-A/14726: Polycomb-Associated Non-Coding RNAs.
Biochemical and Signaling Pathways
BioCyc Knowledge Library PWY-7919 - protein N-glycosylation processing phase (mammalian)
BioCarta from NCI Cancer Genome Anatomy Project h_eradPathway - ER¿associated degradation (ERAD) Pathway
Reactome (by CSHL, EBI, and GO)
Protein Q13724 (Reactome details) participates in the following event(s):
R-HSA-532678 Trimming of the first glucose by by mannosyl-oligosaccharide glucosidase R-HSA-532668 N-glycan trimming in the ER and Calnexin/Calreticulin cycle R-HSA-446203 Asparagine N-linked glycosylation R-HSA-597592 Post-translational protein modification R-HSA-392499 Metabolism of proteins
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
