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Guidelines

MIRAGE

mannosidase alpha class 2B member 1

Summary

Gene Symbol

MAN2B1

Aliases

LAMAN
lysosomal alpha-mannosidase

Organism

Homo sapiens (human)

External Links

NCBI Gene

4125

HGNC

6826

KEGG Gene ID

["hsa:4125"]

PubChem

4125

Alliance of Genome Resources

HGNC:6826

JoGo

MAN2B1

TogoVar

MAN2B1

Annotation

Keyword

Alternative splicing
Direct protein sequencing
Disease variant
Disulfide bond
Glycoprotein
Glycosidase
Lysosome
Metal-binding
Proteomics identification
Reference proteome
Secreted
Signal
Zinc
Zymogen

Proteins

Displaying **all 2** entries
UniProt	Protein Name
O00754	Lysosomal acid alpha-mannosidase Mannosidase alpha class 2B member 1 Mannosidase alpha-B
A8K6A7

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 5** entries
GO Term	Evidence Code	PMID
carbohydrate metabolic process	IEA
mannose metabolic process	IEA
glycoprotein catabolic process	IC
glycoprotein catabolic process	IDA
learning or memory

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

catabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process

carbohydrate derivative catabolic process [inference]

glycoprotein catabolic process

macromolecule metabolic process [inference]

macromolecule catabolic process [inference]

protein catabolic process [inference]

glycoprotein catabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

glycoprotein catabolic process

protein catabolic process [inference]

glycoprotein catabolic process

primary metabolic process [inference]

carbohydrate metabolic process

monosaccharide metabolic process [inference]

hexose metabolic process [inference]

mannose metabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

glycoprotein catabolic process

protein catabolic process [inference]

glycoprotein catabolic process

small molecule metabolic process [inference]

monosaccharide metabolic process [inference]

hexose metabolic process [inference]

mannose metabolic process

carbohydrate derivative metabolic process [inference]

glycoprotein metabolic process [inference]

glycoprotein catabolic process

carbohydrate derivative catabolic process [inference]

glycoprotein catabolic process

multicellular organismal process [inference]

system process [inference]

nervous system process [inference]

cognition [inference]

learning or memory

behavior [inference]

learning or memory

Displaying entries **1 - 5** of 12 in total

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GO Term	Evidence Code	PMID
extracellular region	EXP
extracellular region	TAS
extracellular region	IDA
extracellular region	IEA
obsolete extracellular space

GO Hierarchy

cellular component

cellular anatomical structure [inference]

membrane-enclosed lumen [inference]

organelle lumen [inference]

vesicle lumen [inference]

cytoplasmic vesicle lumen [inference]

secretory granule lumen [inference]

azurophil granule lumen

intracellular organelle lumen [inference]

vacuolar lumen [inference]

cytoplasmic vesicle lumen [inference]

secretory granule lumen [inference]

azurophil granule lumen

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular membrane-bounded organelle [inference]

vacuole [inference]

lytic vacuole [inference]

lysosome

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

vacuole [inference]

lytic vacuole [inference]

lysosome

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

Displaying entries **1 - 5** of 6 in total

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GO Term	Evidence Code	PMID
alpha-mannosidase activity	EXP
alpha-mannosidase activity	IDA
alpha-mannosidase activity	IBA
alpha-mannosidase activity	IEA
carbohydrate binding	IEA

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

hydrolase activity, acting on glycosyl bonds [inference]

hydrolase activity, hydrolyzing O-glycosyl compounds [inference]

mannosidase activity [inference]

alpha-mannosidase activity

binding [inference]

carbohydrate binding

small molecule binding [inference]

ion binding [inference]

cation binding [inference]

metal ion binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Alpha mannosidase

Functional Category

G: Carbohydrate transport and metabolism
J: Translation, ribosomal structure and biogenesis

Displaying **all 6** entries
Gene Ontology
alpha-mannosidase activity
catalytic activity
hydrolase activity, acting on glycosyl bonds
hydrolase activity
mannose metabolic process
metal ion binding

Displaying **all 9** entries
InterPro
Galactose mutarotase-like domain superfamily
Glycoside hydrolase 38, N-terminal domain superfamily
Glycoside hydrolase families 57/38, central domain superfamily
Glycoside hydrolase family 38, N-terminal domain
Glycoside hydrolase family 38, central domain superfamily
Glycoside hydrolase family 38, central domain
Glycoside hydrolase/deacetylase, beta/alpha-barrel
Glycosyl hydrolase family 38, C-terminal
Glycosyl hydrolase, all-beta

Human Protein Atlas

ENSG00000104774

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K12311

Name

lysosomal alpha-mannosidase [EC:3.2.1.24]

References

19722277

Reactions

Displaying **all 2** entries
KEGG Reaction	Enzyme	Acceptor	Product
R08717	3.2.1.24	G88469YE	G47023PF
R08718	3.2.1.24	G31265VL	G24464VD

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:3413	alpha-mannosidosis	Glyco-Disease Genes Database Glyco-Disease Genes Database Glyco-Disease Genes Database Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **1 - 10** of **126** in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000010	Recurrent urinary tract infections
HP:0000023	Inguinal hernia
HP:0000158	Macroglossia
HP:0000212	Gingival overgrowth
HP:0000248	Brachycephaly
HP:0000256	Macrocephaly
HP:0000272	Malar flattening
HP:0000280	Coarse facial features
HP:0000286	Epicanthus

Displaying **all 3** entries
Disease ID	Disease Name
ORPHA:309282	alpha-mannosidosis, infantile form
ORPHA:309288	alpha-mannosidosis, adult form
OMIM:248500	alpha-mannosidosis

PubChem Disease

GHR Health Conditions

Alpha-mannosidosis

MedGen Diseases

Deficiency of alpha-mannosidase

OMIM Phenotypes

MANNOSIDOSIS, ALPHA B, LYSOSOMAL; MANSA

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

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Acknowledgements

Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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GlyCosmos Portal v4.5.0

Last updated: April 6, 2026