Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Alpha-2-macroglobulin receptor-associated protein

Gene

LRPAP1

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

Functioni

Molecular chaperone for LDL receptor-related proteins that may regulate their ligand binding activity along the secretory pathway.1 Publication

GO - Molecular functioni

  • amyloid-beta binding Source: ARUK-UCL
  • heparin binding Source: UniProtKB-KW
  • lipase binding Source: Ensembl
  • low-density lipoprotein particle receptor binding Source: UniProtKB
  • receptor antagonist activity Source: BHF-UCL
  • signaling receptor binding Source: ParkinsonsUK-UCL
  • very-low-density lipoprotein particle receptor binding Source: BHF-UCL

GO - Biological processi

Keywordsi

Molecular functionHeparin-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Alpha-2-macroglobulin receptor-associated protein
Short name:
Alpha-2-MRAP
Alternative name(s):
Low density lipoprotein receptor-related protein-associated protein 1
Short name:
RAP
Gene namesi
Name:LRPAP1
Synonyms:A2MRAP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 4

Organism-specific databases

EuPathDBiHostDB:ENSG00000163956.10
HGNCiHGNC:6701 LRPAP1
MIMi104225 gene
neXtProtiNX_P30533

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

Keywords - Cellular componenti

Endoplasmic reticulum, Endosome, Golgi apparatus

Pathology & Biotechi

Involvement in diseasei

Myopia 23, autosomal recessive (MYP23)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA refractive error of the eye, in which parallel rays from a distant object come to focus in front of the retina, vision being better for near objects than for far.
See also OMIM:615431

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi283H → A: Strongly reduced interaction with LRP1; when associated with A-291; A-293; A-302; A-307 and A-341. 1 Publication1
Mutagenesisi291H → A: Strongly reduced interaction with LRP1; when associated with A-283; A-293; A-302; A-307 and A-341. 1 Publication1
Mutagenesisi293H → A: Strongly reduced interaction with LRP1; when associated with A-283; A-291; A-302; A-307 and A-341. 1 Publication1
Mutagenesisi302H → A: Strongly reduced interaction with LRP1; when associated with A-283; A-291; A-293; A-307 and A-341. 1 Publication1
Mutagenesisi307H → A: Strongly reduced interaction with LRP1; when associated with A-283; A-291; A-293; A-302 and A-341. 1 Publication1
Mutagenesisi341H → A: Strongly reduced interaction with LRP1; when associated with A-283; A-291; A-293; A-302 and A-307. 1 Publication1

Organism-specific databases

DisGeNETi4043
MalaCardsiLRPAP1
MIMi615431 phenotype
OpenTargetsiENSG00000163956
Orphaneti98619 Rare isolated myopia
PharmGKBiPA30458

Polymorphism and mutation databases

BioMutaiLRPAP1
DMDMi231539

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 34Sequence analysisAdd BLAST34
ChainiPRO_000002072435 – 357Alpha-2-macroglobulin receptor-associated proteinAdd BLAST323

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei50PhosphoserineBy similarity1
Modified residuei135PhosphoserineBy similarity1
Modified residuei248PhosphothreonineCombined sources1
Glycosylationi268N-linked (GlcNAc...) asparagine1 Publication1

Post-translational modificationi

N-glycosylated.3 Publications

Keywords - PTMi

Glycoprotein, Phosphoprotein

Proteomic databases

EPDiP30533
MaxQBiP30533
PaxDbiP30533
PeptideAtlasiP30533
PRIDEiP30533
ProteomicsDBi54716
TopDownProteomicsiP30533

PTM databases

iPTMnetiP30533
PhosphoSitePlusiP30533

Expressioni

Gene expression databases

BgeeiENSG00000163956 Expressed in 233 organ(s), highest expression level in caudate nucleus
CleanExiHS_LRPAP1
ExpressionAtlasiP30533 baseline and differential
GenevisibleiP30533 HS

Organism-specific databases

HPAiCAB025518
HPA008001

Interactioni

Subunit structurei

Interacts with the LRP1/alpha-2-macroglobulin receptor heavy and light chains; the interaction is transient and coincides with a reduction of ligand binding by the receptor (PubMed:1712782, PubMed:1400426, PubMed:7774585, PubMed:16938309, PubMed:16678114). Interacts with LRP2/glycoprotein 330 (PubMed:1400426). Interacts with LRP1B; binding is followed by internalization and degradation (PubMed:11384978). Interacts with LDLR (PubMed:16630895).7 Publications

Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

BioGridi110221, 24 interactors
DIPiDIP-39355N
IntActiP30533, 18 interactors
MINTiP30533
STRINGi9606.ENSP00000421922

Structurei

Secondary structure

1357
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

ProteinModelPortaliP30533
SMRiP30533
ModBaseiSearch...
MobiDBiSearch...

Miscellaneous databases

EvolutionaryTraceiP30533

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni237 – 353LDL receptor bindingSequence analysisAdd BLAST117

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili219 – 310Sequence analysisAdd BLAST92

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi354 – 357Prevents secretion from ER1 Publication4

Sequence similaritiesi

Belongs to the alpha-2-MRAP family.Curated

Keywords - Domaini

Coiled coil, Signal

Phylogenomic databases

eggNOGiKOG3956 Eukaryota
ENOG410YJFP LUCA
GeneTreeiENSGT00390000004855
HOGENOMiHOG000033926
HOVERGENiHBG000197
InParanoidiP30533
KOiK22290
OMAiNQVWEKA
OrthoDBiEOG091G0EO4
PhylomeDBiP30533
TreeFamiTF320678

Family and domain databases

CDDicd14806 RAP_D1, 1 hit
cd14807 RAP_D2, 1 hit
cd14808 RAP_D3, 1 hit
Gene3Di1.20.81.10, 3 hits
InterProiView protein in InterPro
IPR038003 A2-macroglobuin_RAP
IPR010483 Alpha_2_MRAP_C
IPR009066 MG_RAP_rcpt_1
IPR038001 RAP_D2
IPR037999 RAP_D3
IPR036744 RAP_sf
PANTHERiPTHR16560 PTHR16560, 1 hit
PfamiView protein in Pfam
PF06401 Alpha-2-MRAP_C, 1 hit
PF06400 Alpha-2-MRAP_N, 1 hit
SUPFAMiSSF47045 SSF47045, 3 hits
PROSITEiView protein in PROSITE
PS00014 ER_TARGET, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.iShow all

P30533-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAPRRVRSFL RGLPALLLLL LFLGPWPAAS HGGKYSREKN QPKPSPKRES
60 70 80 90 100
GEEFRMEKLN QLWEKAQRLH LPPVRLAELH ADLKIQERDE LAWKKLKLDG
110 120 130 140 150
LDEDGEKEAR LIRNLNVILA KYGLDGKKDA RQVTSNSLSG TQEDGLDDPR
160 170 180 190 200
LEKLWHKAKT SGKFSGEELD KLWREFLHHK EKVHEYNVLL ETLSRTEEIH
210 220 230 240 250
ENVISPSDLS DIKGSVLHSR HTELKEKLRS INQGLDRLRR VSHQGYSTEA
260 270 280 290 300
EFEEPRVIDL WDLAQSANLT DKELEAFREE LKHFEAKIEK HNHYQKQLEI
310 320 330 340 350
AHEKLRHAES VGDGERVSRS REKHALLEGR TKELGYTVKK HLQDLSGRIS

RARHNEL
Length:357
Mass (Da):41,466
Last modified:April 1, 1993 - v1
Checksum:iD56A7DA754125CFA
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
D6REW6D6REW6_HUMAN
Alpha-2-macroglobulin receptor-asso...
LRPAP1
105Annotation score:

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti100G → D in BAD96247 (Ref. 3) Curated1
Sequence conflicti183V → A in BAD96224 (Ref. 3) Curated1
Sequence conflicti339K → M in BAD96247 (Ref. 3) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_050660114N → S. Corresponds to variant dbSNP:rs2228158Ensembl.1
Natural variantiVAR_011821311V → M1 PublicationCorresponds to variant dbSNP:rs1800493Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M63959 mRNA Translation: AAA51553.1
AF035767
, AF035760, AF035761, AF035762, AF035763, AF035764, AF035765, AF035766 Genomic DNA Translation: AAC67373.1
AK222504 mRNA Translation: BAD96224.1
AK222527 mRNA Translation: BAD96247.1
AL590235 Genomic DNA No translation available.
CH471131 Genomic DNA Translation: EAW82460.1
CH471131 Genomic DNA Translation: EAW82461.1
BC105074 mRNA Translation: AAI05075.1
BC112067 mRNA Translation: AAI12068.1
U06976 Genomic DNA Translation: AAA87889.1
CCDSiCCDS3371.1
PIRiA39875
RefSeqiNP_002328.1, NM_002337.3
UniGeneiHs.40966

Genome annotation databases

EnsembliENST00000500728; ENSP00000421922; ENSG00000163956
GeneIDi4043
KEGGihsa:4043
UCSCiuc003ghh.5 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiAMRP_HUMAN
AccessioniPrimary (citable) accession number: P30533
Secondary accession number(s): D3DVR9
, Q2M310, Q53HQ3, Q53HS6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 1, 1993
Last sequence update: April 1, 1993
Last modified: September 12, 2018
This is version 178 of the entry and version 1 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
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again