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Protein

Lysosome-associated membrane glycoprotein 2

Gene

LAMP2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Plays an important role in chaperone-mediated autophagy, a process that mediates lysosomal degradation of proteins in response to various stresses and as part of the normal turnover of proteins with a long biological half-live (PubMed:8662539, PubMed:11082038, PubMed:18644871, PubMed:24880125, PubMed:27628032). Functions by binding target proteins, such as GAPDH and MLLT11, and targeting them for lysosomal degradation (PubMed:8662539, PubMed:11082038, PubMed:18644871, PubMed:24880125). Plays a role in lysosomal protein degradation in response to starvation (By similarity). Required for the fusion of autophagosomes with lysosomes during autophagy (PubMed:27628032). Cells that lack LAMP2 express normal levels of VAMP8, but fail to accumulate STX17 on autophagosomes, which is the most likely explanation for the lack of fusion between autophagosomes and lysosomes (PubMed:27628032). Required for normal degradation of the contents of autophagosomes (PubMed:27628032). Required for efficient MHCII-mediated presentation of exogenous antigens via its function in lysosomal protein degradation; antigenic peptides generated by proteases in the endosomal/lysosomal compartment are captured by nascent MHCII subunits (PubMed:20518820). Is not required for efficient MHCII-mediated presentation of endogenous antigens (PubMed:20518820).By similarity6 Publications
Isoform LAMP-2C: Modulates chaperone-mediated autophagy. Decreases presentation of endogenous antigens by MHCII. Does not play a role in the presentation of exogenous and membrane-derived antigens by MHCII.1 Publication

GO - Molecular functioni

  • enzyme binding Source: UniProtKB
  • protein domain specific binding Source: Ensembl

GO - Biological processi

Keywordsi

Biological processAutophagy

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation
R-HSA-6798695 Neutrophil degranulation

Protein family/group databases

TCDBi9.A.16.1.2 the lysosomal protein import (lpi) family

Names & Taxonomyi

Protein namesi
Recommended name:
Lysosome-associated membrane glycoprotein 2
Short name:
LAMP-2
Short name:
Lysosome-associated membrane protein 2
Alternative name(s):
CD107 antigen-like family member B
LGP-961 Publication
CD_antigen: CD107b
Gene namesi
Name:LAMP2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome X

Organism-specific databases

EuPathDBiHostDB:ENSG00000005893.15
HGNCiHGNC:6501 LAMP2
MIMi309060 gene
neXtProtiNX_P13473

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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini29 – 375LumenalSequence analysisAdd BLAST347
Transmembranei376 – 399HelicalPROSITE-ProRule annotationAdd BLAST24
Topological domaini400 – 410CytoplasmicPROSITE-ProRule annotationAdd BLAST11

Keywords - Cellular componenti

Cell membrane, Cytoplasmic vesicle, Endosome, Lysosome, Membrane

Pathology & Biotechi

Involvement in diseasei

Danon disease (DAND)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionDAND is a lysosomal glycogen storage disease characterized by the clinical triad of cardiomyopathy, vacuolar myopathy and mental retardation. It is often associated with an accumulation of glycogen in muscle and lysosomes.
See also OMIM:300257
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_026230321W → R in DAND. 2 PublicationsCorresponds to variant dbSNP:rs104894859EnsemblClinVar.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi401 – 404KHHH → AAAA: Impairs binding and subsequent lysosomal degradation of target proteins, such as GAPDH. 1 Publication4

Keywords - Diseasei

Disease mutation, Glycogen storage disease

Organism-specific databases

DisGeNETi3920
MalaCardsiLAMP2
MIMi300257 phenotype
OpenTargetsiENSG00000005893
Orphaneti34587 Glycogen storage disease due to LAMP-2 deficiency
PharmGKBiPA30285

Polymorphism and mutation databases

BioMutaiLAMP2
DMDMi1708854

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 283 PublicationsAdd BLAST28
ChainiPRO_000001711029 – 410Lysosome-associated membrane glycoprotein 2Add BLAST382

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Glycosylationi32N-linked (GlcNAc...) (polylactosaminoglycan) asparagine1 Publication1
Glycosylationi38N-linked (GlcNAc...) (polylactosaminoglycan) asparagine1 Publication1
Disulfide bondi41 ↔ 79PROSITE-ProRule annotation
Glycosylationi49N-linked (GlcNAc...) asparagine2 Publications1
Glycosylationi58N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi75N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi101N-linked (GlcNAc...) asparagine3 Publications1
Glycosylationi123N-linked (GlcNAc...) asparagine2 Publications1
Disulfide bondi153 ↔ 189PROSITE-ProRule annotation
Glycosylationi179N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi195O-linked (GalNAc...) serine1 Publication1
Glycosylationi196O-linked (GalNAc...) threonine1 Publication1
Glycosylationi200O-linked (GalNAc...) threonine1 Publication1
Glycosylationi203O-linked (GalNAc...) threonine1 Publication1
Glycosylationi204O-linked (GalNAc...) threonine1 Publication1
Glycosylationi207O-linked (GalNAc...) serine; partial1 Publication1
Glycosylationi209O-linked (GalNAc...) threonine; partial1 Publication1
Glycosylationi210O-linked (GalNAc...) threonine1 Publication1
Glycosylationi211O-linked (GalNAc...) threonine1 Publication1
Glycosylationi213O-linked (GalNAc...) threonine; partial1 Publication1
Glycosylationi229N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi232 ↔ 265PROSITE-ProRule annotation
Glycosylationi242N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi257N-linked (GlcNAc...) asparagine2 Publications1
Glycosylationi275N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi300N-linked (GlcNAc...) asparagine1 Publication1
Glycosylationi307N-linked (GlcNAc...) (polylactosaminoglycan) asparagine1 Publication1
Glycosylationi317N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi331 ↔ 368PROSITE-ProRule annotation
Glycosylationi356N-linked (GlcNAc...) asparagine1 Publication1

Post-translational modificationi

O- and N-glycosylated; some of the 16 N-linked glycans are polylactosaminoglycans.6 Publications

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

EPDiP13473
MaxQBiP13473
PeptideAtlasiP13473
PRIDEiP13473
ProteomicsDBi52910
52911 [P13473-2]
52912 [P13473-3]

PTM databases

GlyConnecti356
iPTMnetiP13473
PhosphoSitePlusiP13473
SwissPalmiP13473
UniCarbKBiP13473

Miscellaneous databases

PMAP-CutDBiP13473

Expressioni

Tissue specificityi

Isoform LAMP-2A is highly expressed in placenta, lung and liver, less in kidney and pancreas, low in brain and skeletal muscle (PubMed:7488019, PubMed:26856698). Isoform LAMP-2B is detected in spleen, thymus, prostate, testis, small intestine, colon, skeletal muscle, brain, placenta, lung, kidney, ovary and pancreas and liver (PubMed:7488019, PubMed:26856698). Isoform LAMP-2C is detected in small intestine, colon, heart, brain, skeletal muscle, and at lower levels in kidney and placenta (PubMed:26856698).2 Publications

Inductioni

In peripheral blood B cells isoform LAMP-2A, LAMP-2B and LAMP-2C are up-regulated in response to treatments that stimulate immune responses via the Toll-like receptors TLR7 or TLR9.1 Publication

Gene expression databases

BgeeiENSG00000005893 Expressed in 236 organ(s), highest expression level in corpus callosum
CleanExiHS_LAMP2
ExpressionAtlasiP13473 baseline and differential
GenevisibleiP13473 HS

Organism-specific databases

HPAiCAB005272
HPA029100

Interactioni

Subunit structurei

Monomer (PubMed:18644871, PubMed:25342746). Homodimer (PubMed:25342746). Homotrimer (PubMed:25342746). Forms large homooligomers (PubMed:18644871). Interacts (via its cytoplasmic region) with HSPA8 (PubMed:25342746). Interacts with HSP90 in the lysosome lumen; this enhances LAMP2 stability (By similarity). Interacts with MLLT11 (PubMed:24880125).By similarity3 Publications

GO - Molecular functioni

Protein-protein interaction databases

BioGridi110114, 96 interactors
IntActiP13473, 17 interactors
MINTiP13473

Structurei

Secondary structure

1410
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP13473
SMRiP13473
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni29 – 192First lumenal domainAdd BLAST164
Regioni193 – 228HingeAdd BLAST36
Regioni229 – 375Second lumenal domainAdd BLAST147
Regioni401 – 404Important for binding and subsequent lysosomal degradation of target proteins1 Publication4

Sequence similaritiesi

Belongs to the LAMP family.PROSITE-ProRule annotation

Keywords - Domaini

Signal, Transmembrane, Transmembrane helix

Phylogenomic databases

GeneTreeiENSGT00530000063068
HOGENOMiHOG000230942
HOVERGENiHBG052303
InParanoidiP13473
KOiK06528
OMAiTGSCHPQ
OrthoDBiEOG091G09EV
PhylomeDBiP13473
TreeFamiTF316339

Family and domain databases

InterProiView protein in InterPro
IPR018134 LAMP_CS
IPR002000 Lysosome-assoc_membr_glycop
PANTHERiPTHR11506 PTHR11506, 1 hit
PfamiView protein in Pfam
PF01299 Lamp, 1 hit
PRINTSiPR00336 LYSASSOCTDMP
PROSITEiView protein in PROSITE
PS00310 LAMP_1, 1 hit
PS00311 LAMP_2, 1 hit
PS51407 LAMP_3, 1 hit

Sequences (3+)i

Sequence statusi: Complete.

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

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 3 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform LAMP-2A (identifier: P13473-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MVCFRLFPVP GSGLVLVCLV LGAVRSYALE LNLTDSENAT CLYAKWQMNF
60 70 80 90 100
TVRYETTNKT YKTVTISDHG TVTYNGSICG DDQNGPKIAV QFGPGFSWIA
110 120 130 140 150
NFTKAASTYS IDSVSFSYNT GDNTTFPDAE DKGILTVDEL LAIRIPLNDL
160 170 180 190 200
FRCNSLSTLE KNDVVQHYWD VLVQAFVQNG TVSTNEFLCD KDKTSTVAPT
210 220 230 240 250
IHTTVPSPTT TPTPKEKPEA GTYSVNNGND TCLLATMGLQ LNITQDKVAS
260 270 280 290 300
VININPNTTH STGSCRSHTA LLRLNSSTIK YLDFVFAVKN ENRFYLKEVN
310 320 330 340 350
ISMYLVNGSV FSIANNNLSY WDAPLGSSYM CNKEQTVSVS GAFQINTFDL
360 370 380 390 400
RVQPFNVTQG KYSTAQDCSA DDDNFLVPIA VGAALAGVLI LVLLAYFIGL
410
KHHHAGYEQF
Length:410
Mass (Da):44,961
Last modified:October 1, 1996 - v2
Checksum:i9E08E3B62D58F454
GO
Isoform LAMP-2B (identifier: P13473-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     367-410: DCSADDDNFL...KHHHAGYEQF → ECSLDDDTIL...RKSYAGYQTL

Show »
Length:410
Mass (Da):44,956
Checksum:i70B523369D40DEFA
GO
Isoform LAMP-2C (identifier: P13473-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     366-410: QDCSADDDNF...KHHHAGYEQF → EECSADSDLN...RKSRTGYQSV

Show »
Length:411
Mass (Da):45,170
Checksum:iB37AAC83BA1C3B1B
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0YCG2H0YCG2_HUMAN
Lysosome-associated membrane glycop...
LAMP2
258Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti8 – 13PVPGSG → RFRSGLR no nucleotide entry (PubMed:8407947).Curated6
Sequence conflicti53R → G no nucleotide entry (PubMed:8407947).Curated1
Sequence conflicti68D → V in AAB41647 (PubMed:8517882).Curated1
Sequence conflicti111I → N in AAB41647 (PubMed:8517882).Curated1
Sequence conflicti143I → Y in AAB41647 (PubMed:8517882).Curated1
Sequence conflicti220A → P in AAB41647 (PubMed:8517882).Curated1
Sequence conflicti234L → R in AAB41647 (PubMed:8517882).Curated1
Sequence conflicti263 – 269GSCRSHT → AAAVSH no nucleotide entry (PubMed:8407947).Curated7
Sequence conflicti322 – 326DAPLG → MPP in AAA60383 (PubMed:3198605).Curated5

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_011992256P → H. Corresponds to variant dbSNP:rs1043878Ensembl.1
Natural variantiVAR_026230321W → R in DAND. 2 PublicationsCorresponds to variant dbSNP:rs104894859EnsemblClinVar.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_042519366 – 410QDCSA…GYEQF → EECSADSDLNFLIPVAVGVA LGFLIIVVFISYMIGRRKSR TGYQSV in isoform LAMP-2C. 1 PublicationAdd BLAST45
Alternative sequenceiVSP_003044367 – 410DCSAD…GYEQF → ECSLDDDTILIPIIVGAGLS GLIIVIVIAYVIGRRKSYAG YQTL in isoform LAMP-2B. 3 PublicationsAdd BLAST44

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04183 mRNA Translation: AAA60383.1
L09717
, L09709, L09710, L09711, L09712, L09713, L09714, L09715, L09716 Genomic DNA Translation: AAB41647.1
X77196 mRNA Translation: CAA54416.1
S79873 mRNA Translation: AAB35426.1
U36336 mRNA Translation: AAA91149.1
AY561849 mRNA Translation: AAS67876.1
AK291090 mRNA Translation: BAF83779.1
AC002476 Genomic DNA Translation: AAB67313.1
AC002476 Genomic DNA Translation: AAB67314.1
CH471107 Genomic DNA Translation: EAX11881.1
CH471107 Genomic DNA Translation: EAX11882.1
CH471107 Genomic DNA Translation: EAX11883.1
CH471107 Genomic DNA Translation: EAX11884.1
BC002965 mRNA Translation: AAH02965.1
CCDSiCCDS14599.1 [P13473-1]
CCDS14600.1 [P13473-2]
CCDS48159.1 [P13473-3]
PIRiJC2414 B31959
JC4317
RefSeqiNP_001116078.1, NM_001122606.1 [P13473-3]
NP_002285.1, NM_002294.2 [P13473-1]
NP_054701.1, NM_013995.2 [P13473-2]
UniGeneiHs.496684

Genome annotation databases

EnsembliENST00000200639; ENSP00000200639; ENSG00000005893 [P13473-1]
ENST00000371335; ENSP00000360386; ENSG00000005893 [P13473-2]
ENST00000434600; ENSP00000408411; ENSG00000005893 [P13473-3]
GeneIDi3920
KEGGihsa:3920
UCSCiuc004ess.5 human [P13473-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
J04183 mRNA Translation: AAA60383.1
L09717
, L09709, L09710, L09711, L09712, L09713, L09714, L09715, L09716 Genomic DNA Translation: AAB41647.1
X77196 mRNA Translation: CAA54416.1
S79873 mRNA Translation: AAB35426.1
U36336 mRNA Translation: AAA91149.1
AY561849 mRNA Translation: AAS67876.1
AK291090 mRNA Translation: BAF83779.1
AC002476 Genomic DNA Translation: AAB67313.1
AC002476 Genomic DNA Translation: AAB67314.1
CH471107 Genomic DNA Translation: EAX11881.1
CH471107 Genomic DNA Translation: EAX11882.1
CH471107 Genomic DNA Translation: EAX11883.1
CH471107 Genomic DNA Translation: EAX11884.1
BC002965 mRNA Translation: AAH02965.1
CCDSiCCDS14599.1 [P13473-1]
CCDS14600.1 [P13473-2]
CCDS48159.1 [P13473-3]
PIRiJC2414 B31959
JC4317
RefSeqiNP_001116078.1, NM_001122606.1 [P13473-3]
NP_002285.1, NM_002294.2 [P13473-1]
NP_054701.1, NM_013995.2 [P13473-2]
UniGeneiHs.496684

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2MOFNMR-A369-410[»]
2MOMNMR-A/B/C369-410[»]
ProteinModelPortaliP13473
SMRiP13473
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi110114, 96 interactors
IntActiP13473, 17 interactors
MINTiP13473

Protein family/group databases

TCDBi9.A.16.1.2 the lysosomal protein import (lpi) family

PTM databases

GlyConnecti356
iPTMnetiP13473
PhosphoSitePlusiP13473
SwissPalmiP13473
UniCarbKBiP13473

Polymorphism and mutation databases

BioMutaiLAMP2
DMDMi1708854

Proteomic databases

EPDiP13473
MaxQBiP13473
PeptideAtlasiP13473
PRIDEiP13473
ProteomicsDBi52910
52911 [P13473-2]
52912 [P13473-3]

Protocols and materials databases

DNASUi3920
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000200639; ENSP00000200639; ENSG00000005893 [P13473-1]
ENST00000371335; ENSP00000360386; ENSG00000005893 [P13473-2]
ENST00000434600; ENSP00000408411; ENSG00000005893 [P13473-3]
GeneIDi3920
KEGGihsa:3920
UCSCiuc004ess.5 human [P13473-1]

Organism-specific databases

CTDi3920
DisGeNETi3920
EuPathDBiHostDB:ENSG00000005893.15
GeneCardsiLAMP2
HGNCiHGNC:6501 LAMP2
HPAiCAB005272
HPA029100
MalaCardsiLAMP2
MIMi300257 phenotype
309060 gene
neXtProtiNX_P13473
OpenTargetsiENSG00000005893
Orphaneti34587 Glycogen storage disease due to LAMP-2 deficiency
PharmGKBiPA30285
GenAtlasiSearch...

Phylogenomic databases

GeneTreeiENSGT00530000063068
HOGENOMiHOG000230942
HOVERGENiHBG052303
InParanoidiP13473
KOiK06528
OMAiTGSCHPQ
OrthoDBiEOG091G09EV
PhylomeDBiP13473
TreeFamiTF316339

Enzyme and pathway databases

ReactomeiR-HSA-114608 Platelet degranulation
R-HSA-6798695 Neutrophil degranulation

Miscellaneous databases

ChiTaRSiLAMP2 human
GeneWikiiLAMP2
GenomeRNAii3920
PMAP-CutDBiP13473
PROiPR:P13473
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000005893 Expressed in 236 organ(s), highest expression level in corpus callosum
CleanExiHS_LAMP2
ExpressionAtlasiP13473 baseline and differential
GenevisibleiP13473 HS

Family and domain databases

InterProiView protein in InterPro
IPR018134 LAMP_CS
IPR002000 Lysosome-assoc_membr_glycop
PANTHERiPTHR11506 PTHR11506, 1 hit
PfamiView protein in Pfam
PF01299 Lamp, 1 hit
PRINTSiPR00336 LYSASSOCTDMP
PROSITEiView protein in PROSITE
PS00310 LAMP_1, 1 hit
PS00311 LAMP_2, 1 hit
PS51407 LAMP_3, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiLAMP2_HUMAN
AccessioniPrimary (citable) accession number: P13473
Secondary accession number(s): A8K4X5
, D3DTF0, Q16641, Q6Q3G8, Q96J30, Q99534, Q9UD93
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 1, 1990
Last sequence update: October 1, 1996
Last modified: November 7, 2018
This is version 199 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Human chromosome X
    Human chromosome X: entries, gene names and cross-references to MIM
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. Human cell differentiation molecules
    CD nomenclature of surface proteins of human leucocytes and list of entries
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