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Protein

Low density lipoprotein receptor adapter protein 1

Gene

Ldlrap1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Adapter protein (clathrin-associated sorting protein (CLASP)) required for efficient endocytosis of the LDL receptor (LDLR) in polarized cells such as hepatocytes and lymphocytes, but not in non-polarized cells (fibroblasts). May be required for LDL binding and internalization but not for receptor clustering in coated pits. May facilitate the endocytocis of LDLR and LDLR-LDL complexes from coated pits by stabilizing the interaction between the receptor and the structural components of the pits. May also be involved in the internalization of other LDLR family members. Binds to phosphoinositides, which regulate clathrin bud assembly at the cell surface.2 Publications

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Biological processi

Cholesterol metabolism, Endocytosis, Lipid metabolism, Steroid metabolism, Sterol metabolism

Enzyme and pathway databases

ReactomeiR-MMU-171052. LDL-mediated lipid transport.
R-MMU-8856825. Cargo recognition for clathrin-mediated endocytosis.
R-MMU-8856828. Clathrin-mediated endocytosis.

Names & Taxonomyi

Protein namesi
Recommended name:
Low density lipoprotein receptor adapter protein 1Curated
Alternative name(s):
Autosomal recessive hypercholesterolemia protein homolog1 Publication
Gene namesi
Name:Ldlrap1Imported
Synonyms:Arh1 Publication
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 4

Organism-specific databases

MGIiMGI:2140175. Ldlrap1.

Subcellular locationi

GO - Cellular componenti

  • AP-1 adaptor complex Source: Ensembl
  • AP-2 adaptor complex Source: Ensembl
  • axon Source: Ensembl
  • basal plasma membrane Source: UniProtKB
  • cytoplasm Source: BHF-UCL
  • cytoplasmic side of plasma membrane Source: MGI
  • cytosol Source: UniProtKB
  • early endosome Source: MGI
  • neurofilament Source: BHF-UCL
  • recycling endosome Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm

Pathology & Biotechi

Disruption phenotypei

Mice are extremely sensitive to cholesterol intake. LDLR are expressed at normal levels, but are sequestered at the hepatocyte surface. LDL internalization defect is caused by the inability of the LDLR to enter the endocytic cycle.1 Publication

Keywords - Diseasei

Atherosclerosis, Hyperlipidemia

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00000646761 – 308Low density lipoprotein receptor adapter protein 1Add BLAST308

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineBy similarity1
Modified residuei14PhosphoserineBy similarity1
Modified residuei201PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ8C142.
MaxQBiQ8C142.
PaxDbiQ8C142.
PeptideAtlasiQ8C142.
PRIDEiQ8C142.

PTM databases

iPTMnetiQ8C142.
PhosphoSitePlusiQ8C142.

Expressioni

Gene expression databases

BgeeiENSMUSG00000037295.
GenevisibleiQ8C142. MM.

Interactioni

Subunit structurei

Interacts (via PID domain) with LDLR (via NPXY motif). Binds to soluble clathrin trimers. Interacts with AP2B1; the interaction mediates the association with the AP-2 complex (By similarity). Interacts with VLDLR (PubMed:12746448).By similarity1 Publication

GO - Molecular functioni

Protein-protein interaction databases

BioGridi221366. 2 interactors.
STRINGi10090.ENSMUSP00000036749.

Structurei

3D structure databases

ProteinModelPortaliQ8C142.
SMRiQ8C142.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini41 – 195PIDPROSITE-ProRule annotationAdd BLAST155

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni248 – 275AP-2 complex bindingBy similarityAdd BLAST28

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi211 – 215Clathrin boxBy similarity5
Motifi256 – 265[DE]-X(1,2)-F-X-X-[FL]-X-X-X-R motifBy similarity10

Domaini

The [DE]-X(1,2)-F-X-X-[FL]-X-X-X-R motif mediates interaction the AP-2 complex subunit AP2B1.By similarity
The PID domain mediates interaction with the NPXY internalization motif of LDLR.By similarity

Sequence similaritiesi

Contains 1 PID domain.PROSITE-ProRule annotation

Phylogenomic databases

eggNOGiKOG3536. Eukaryota.
ENOG410ZHJT. LUCA.
GeneTreeiENSGT00530000062937.
HOGENOMiHOG000030906.
HOVERGENiHBG058060.
InParanoidiQ8C142.
KOiK12474.
OMAiAQDIHYA.
OrthoDBiEOG091G0TOH.
TreeFamiTF314159.

Family and domain databases

Gene3Di2.30.29.30. 1 hit.
InterProiIPR011993. PH_dom-like.
IPR006020. PTB/PI_dom.
[Graphical view]
PfamiPF00640. PID. 1 hit.
[Graphical view]
SMARTiSM00462. PTB. 1 hit.
[Graphical view]
SUPFAMiSSF50729. SSF50729. 1 hit.
PROSITEiPS01179. PID. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q8C142-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MDALKSAGRA LIRSPSLAKQ SWAGGRHRKL PENWTDTRET LLEGMVFSLK
60 70 80 90 100
YLGMTLVERP KGEELSAAAV KRIVATAKAS GKKLQKVTLK VSPRGIILTD
110 120 130 140 150
SLTSQLIENV SIYRISYCTA DKMHDKVFAY IAQSQQNESL ECHAFLCTKR
160 170 180 190 200
KVAQAVTLTV AQAFKVAFEF WQVSKEEKEK REKANQEGGD VPGTRRDSTP
210 220 230 240 250
SLKTLVATGN LLDLEEVAKA PLSTVSANTN NVDETPRPQV LGNNSVVWEL
260 270 280 290 300
DDGLDEAFSR LAQSRTNPQV LDTGLSAQDI HYAQCLSPTD WDKPDSSGID

QDDDVFTF
Length:308
Mass (Da):33,975
Last modified:July 27, 2011 - v3
Checksum:i4183DA9594FD1F2C
GO

Sequence cautioni

The sequence AAH21467 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti260R → K in BAC26238 (PubMed:16141072).Curated1
Sequence conflicti296S → G in AAH66808 (PubMed:15489334).Curated1
Sequence conflicti296S → G in AAH67411 (PubMed:15489334).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK029008 mRNA. Translation: BAC26238.1.
AK148340 mRNA. Translation: BAE28493.1.
AK159800 mRNA. Translation: BAE35380.1.
AK167290 mRNA. Translation: BAE39396.1.
AL645531 Genomic DNA. Translation: CAM17037.1.
CH466552 Genomic DNA. Translation: EDL30001.1.
BC021467 mRNA. Translation: AAH21467.1. Different initiation.
BC066808 mRNA. Translation: AAH66808.1.
BC067411 mRNA. Translation: AAH67411.1.
CCDSiCCDS51327.1.
RefSeqiNP_663529.2. NM_145554.2.
XP_006538486.1. XM_006538423.3.
UniGeneiMm.27486.
Mm.482148.

Genome annotation databases

EnsembliENSMUST00000037828; ENSMUSP00000036749; ENSMUSG00000037295.
GeneIDi100017.
KEGGimmu:100017.
UCSCiuc012dmx.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK029008 mRNA. Translation: BAC26238.1.
AK148340 mRNA. Translation: BAE28493.1.
AK159800 mRNA. Translation: BAE35380.1.
AK167290 mRNA. Translation: BAE39396.1.
AL645531 Genomic DNA. Translation: CAM17037.1.
CH466552 Genomic DNA. Translation: EDL30001.1.
BC021467 mRNA. Translation: AAH21467.1. Different initiation.
BC066808 mRNA. Translation: AAH66808.1.
BC067411 mRNA. Translation: AAH67411.1.
CCDSiCCDS51327.1.
RefSeqiNP_663529.2. NM_145554.2.
XP_006538486.1. XM_006538423.3.
UniGeneiMm.27486.
Mm.482148.

3D structure databases

ProteinModelPortaliQ8C142.
SMRiQ8C142.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi221366. 2 interactors.
STRINGi10090.ENSMUSP00000036749.

PTM databases

iPTMnetiQ8C142.
PhosphoSitePlusiQ8C142.

Proteomic databases

EPDiQ8C142.
MaxQBiQ8C142.
PaxDbiQ8C142.
PeptideAtlasiQ8C142.
PRIDEiQ8C142.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000037828; ENSMUSP00000036749; ENSMUSG00000037295.
GeneIDi100017.
KEGGimmu:100017.
UCSCiuc012dmx.1. mouse.

Organism-specific databases

CTDi26119.
MGIiMGI:2140175. Ldlrap1.

Phylogenomic databases

eggNOGiKOG3536. Eukaryota.
ENOG410ZHJT. LUCA.
GeneTreeiENSGT00530000062937.
HOGENOMiHOG000030906.
HOVERGENiHBG058060.
InParanoidiQ8C142.
KOiK12474.
OMAiAQDIHYA.
OrthoDBiEOG091G0TOH.
TreeFamiTF314159.

Enzyme and pathway databases

ReactomeiR-MMU-171052. LDL-mediated lipid transport.
R-MMU-8856825. Cargo recognition for clathrin-mediated endocytosis.
R-MMU-8856828. Clathrin-mediated endocytosis.

Miscellaneous databases

PROiQ8C142.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000037295.
GenevisibleiQ8C142. MM.

Family and domain databases

Gene3Di2.30.29.30. 1 hit.
InterProiIPR011993. PH_dom-like.
IPR006020. PTB/PI_dom.
[Graphical view]
PfamiPF00640. PID. 1 hit.
[Graphical view]
SMARTiSM00462. PTB. 1 hit.
[Graphical view]
SUPFAMiSSF50729. SSF50729. 1 hit.
PROSITEiPS01179. PID. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiARH_MOUSE
AccessioniPrimary (citable) accession number: Q8C142
Secondary accession number(s): Q3TW86, Q6NWV6, Q8VDQ0
Entry historyi
Integrated into UniProtKB/Swiss-Prot: August 30, 2005
Last sequence update: July 27, 2011
Last modified: November 30, 2016
This is version 120 of the entry and version 3 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.