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Protein

Low density lipoprotein receptor adapter protein 1

Gene

Ldlrap1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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. Required for trafficking of LRP2 to the endocytic recycling compartment which is necessary for LRP2 proteolysis, releasing a tail fragment which translocates to the nucleus and mediates transcriptional repression (By similarity).By similarity2 Publications

GO - Molecular functioni

GO - Biological processi

Keywordsi

Biological processCholesterol metabolism, Endocytosis, Lipid metabolism, Steroid metabolism, Sterol metabolism

Enzyme and pathway databases

ReactomeiR-MMU-8856825 Cargo recognition for clathrin-mediated endocytosis
R-MMU-8856828 Clathrin-mediated endocytosis
R-MMU-8964026 Chylomicron clearance
R-MMU-8964038 LDL clearance

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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 4

Organism-specific databases

MGIiMGI:2140175 Ldlrap1

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

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 residuei198PhosphoserineBy similarity1
Modified residuei201PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ8C142
MaxQBiQ8C142
PaxDbiQ8C142
PeptideAtlasiQ8C142
PRIDEiQ8C142

PTM databases

iPTMnetiQ8C142
PhosphoSitePlusiQ8C142

Expressioni

Gene expression databases

BgeeiENSMUSG00000037295 Expressed in 197 organ(s), highest expression level in ear
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). Interacts with LRP2 (By similarity).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

Phylogenomic databases

eggNOGiKOG3536 Eukaryota
ENOG410ZHJT LUCA
GeneTreeiENSGT00530000062937
HOGENOMiHOG000030906
HOVERGENiHBG058060
InParanoidiQ8C142
KOiK12474
OMAiDIHYAQC
OrthoDBiEOG091G0TOH
TreeFamiTF314159

Family and domain databases

Gene3Di2.30.29.30, 1 hit
InterProiView protein in InterPro
IPR011993 PH-like_dom_sf
IPR006020 PTB/PI_dom
PfamiView protein in Pfam
PF00640 PID, 1 hit
SMARTiView protein in SMART
SM00462 PTB, 1 hit
PROSITEiView protein in PROSITE
PS01179 PID, 1 hit

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

Similar proteinsi

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
OMAiDIHYAQC
OrthoDBiEOG091G0TOH
TreeFamiTF314159

Enzyme and pathway databases

ReactomeiR-MMU-8856825 Cargo recognition for clathrin-mediated endocytosis
R-MMU-8856828 Clathrin-mediated endocytosis
R-MMU-8964026 Chylomicron clearance
R-MMU-8964038 LDL clearance

Miscellaneous databases

PROiPR:Q8C142
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000037295 Expressed in 197 organ(s), highest expression level in ear
GenevisibleiQ8C142 MM

Family and domain databases

Gene3Di2.30.29.30, 1 hit
InterProiView protein in InterPro
IPR011993 PH-like_dom_sf
IPR006020 PTB/PI_dom
PfamiView protein in Pfam
PF00640 PID, 1 hit
SMARTiView protein in SMART
SM00462 PTB, 1 hit
PROSITEiView protein in PROSITE
PS01179 PID, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiARH_MOUSE
AccessioniPrimary (citable) accession number: Q8C142
Secondary accession number(s): Q3TW86, Q6NWV6, Q8VDQ0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 30, 2005
Last sequence update: July 27, 2011
Last modified: November 7, 2018
This is version 134 of the entry and version 3 of the sequence. See 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
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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