Gene/Proteome Database (LMPD)

LMPD ID
LMP006691
Gene ID
4038
Species
Homo sapiens (Human)
Gene Name
low density lipoprotein receptor-related protein 4
Gene Symbol
LRP4
Synonyms
CLSS; LRP-4; LRP10; MEGF7; SOST2
Alternate Names
low-density lipoprotein receptor-related protein 4; multiple epidermal growth factor-like domains 7
Chromosome
11
Map Location
11p11.2
Summary
This gene encodes a member of the low-density lipoprotein receptor-related protein family. The encoded protein may be a regulator of Wnt signaling. Mutations in this gene are associated with Cenani-Lenz syndrome. [provided by RefSeq, May 2010]
Orthologs
View orthologs and multiple alignments for LRP4

Proteins

low-density lipoprotein receptor-related protein 4 precursor
Refseq ID NP_002325
Protein GI 157384998
UniProt ID O75096
mRNA ID NM_002334
Length 1905
RefSeq Status REVIEWED
MRRQWGALLLGALLCAHGLASSPECACGRSHFTCAVSALGECTCIPAQWQCDGDNDCGDHSDEDGCILPTCSPLDFHCDNGKCIRRSWVCDGDNDCEDDSDEQDCPPRECEEDEFPCQNGYCIRSLWHCDGDNDCGDNSDEQCDMRKCSDKEFRCSDGSCIAEHWYCDGDTDCKDGSDEENCPSAVPAPPCNLEEFQCAYGRCILDIYHCDGDDDCGDWSDESDCSSHQPCRSGEFMCDSGLCINAGWRCDGDADCDDQSDERNCTTSMCTAEQFRCHSGRCVRLSWRCDGEDDCADNSDEENCENTGSPQCALDQFLCWNGRCIGQRKLCNGVNDCGDNSDESPQQNCRPRTGEENCNVNNGGCAQKCQMVRGAVQCTCHTGYRLTEDGHTCQDVNECAEEGYCSQGCTNSEGAFQCWCETGYELRPDRRSCKALGPEPVLLFANRIDIRQVLPHRSEYTLLLNNLENAIALDFHHRRELVFWSDVTLDRILRANLNGSNVEEVVSTGLESPGGLAVDWVHDKLYWTDSGTSRIEVANLDGAHRKVLLWQNLEKPRAIALHPMEGTIYWTDWGNTPRIEASSMDGSGRRIIADTHLFWPNGLTIDYAGRRMYWVDAKHHVIERANLDGSHRKAVISQGLPHPFAITVFEDSLYWTDWHTKSINSANKFTGKNQEIIRNKLHFPMDIHTLHPQRQPAGKNRCGDNNGGCTHLCLPSGQNYTCACPTGFRKISSHACAQSLDKFLLFARRMDIRRISFDTEDLSDDVIPLADVRSAVALDWDSRDDHVYWTDVSTDTISRAKWDGTGQEVVVDTSLESPAGLAIDWVTNKLYWTDAGTDRIEVANTDGSMRTVLIWENLDRPRDIVVEPMGGYMYWTDWGASPKIERAGMDASGRQVIISSNLTWPNGLAIDYGSQRLYWADAGMKTIEFAGLDGSKRKVLIGSQLPHPFGLTLYGERIYWTDWQTKSIQSADRLTGLDRETLQENLENLMDIHVFHRRRPPVSTPCAMENGGCSHLCLRSPNPSGFSCTCPTGINLLSDGKTCSPGMNSFLIFARRIDIRMVSLDIPYFADVVVPINITMKNTIAIGVDPQEGKVYWSDSTLHRISRANLDGSQHEDIITTGLQTTDGLAVDAIGRKVYWTDTGTNRIEVGNLDGSMRKVLVWQNLDSPRAIVLYHEMGFMYWTDWGENAKLERSGMDGSDRAVLINNNLGWPNGLTVDKASSQLLWADAHTERIEAADLNGANRHTLVSPVQHPYGLTLLDSYIYWTDWQTRSIHRADKGTGSNVILVRSNLPGLMDMQAVDRAQPLGFNKCGSRNGGCSHLCLPRPSGFSCACPTGIQLKGDGKTCDPSPETYLLFSSRGSIRRISLDTSDHTDVHVPVPELNNVISLDYDSVDGKVYYTDVFLDVIRRADLNGSNMETVIGRGLKTTDGLAVDWVARNLYWTDTGRNTIEASRLDGSCRKVLINNSLDEPRAIAVFPRKGYLFWTDWGHIAKIERANLDGSERKVLINTDLGWPNGLTLDYDTRRIYWVDAHLDRIESADLNGKLRQVLVSHVSHPFALTQQDRWIYWTDWQTKSIQRVDKYSGRNKETVLANVEGLMDIIVVSPQRQTGTNACGVNNGGCTHLCFARASDFVCACPDEPDSRPCSLVPGLVPPAPRATGMSEKSPVLPNTPPTTLYSSTTRTRTSLEEVEGRCSERDARLGLCARSNDAVPAAPGEGLHISYAIGGLLSILLILVVIAALMLYRHKKSKFTDPGMGNLTYSNPSYRTSTQEVKIEAIPKPAMYNQLCYKKEGGPDHNYTKEKIKIVEGICLLSGDDAEWDDLKQLRSSRGGLLRDHVCMKTDTVSIQASSGSLDDTETEQLLQEEQSECSSVHTAATPERRGSLPDTGWKHERKLSSESQV
sig_peptide: 1..20 inference: COORDINATES: ab initio prediction:SignalP:4.0 calculated_mol_wt: 2151 peptide sequence: MRRQWGALLLGALLCAHGLA mat_peptide: 21..1905 product: low-density lipoprotein receptor-related protein 4 calculated_mol_wt: 209914 peptide sequence: SSPECACGRSHFTCAVSALGECTCIPAQWQCDGDNDCGDHSDEDGCILPTCSPLDFHCDNGKCIRRSWVCDGDNDCEDDSDEQDCPPRECEEDEFPCQNGYCIRSLWHCDGDNDCGDNSDEQCDMRKCSDKEFRCSDGSCIAEHWYCDGDTDCKDGSDEENCPSAVPAPPCNLEEFQCAYGRCILDIYHCDGDDDCGDWSDESDCSSHQPCRSGEFMCDSGLCINAGWRCDGDADCDDQSDERNCTTSMCTAEQFRCHSGRCVRLSWRCDGEDDCADNSDEENCENTGSPQCALDQFLCWNGRCIGQRKLCNGVNDCGDNSDESPQQNCRPRTGEENCNVNNGGCAQKCQMVRGAVQCTCHTGYRLTEDGHTCQDVNECAEEGYCSQGCTNSEGAFQCWCETGYELRPDRRSCKALGPEPVLLFANRIDIRQVLPHRSEYTLLLNNLENAIALDFHHRRELVFWSDVTLDRILRANLNGSNVEEVVSTGLESPGGLAVDWVHDKLYWTDSGTSRIEVANLDGAHRKVLLWQNLEKPRAIALHPMEGTIYWTDWGNTPRIEASSMDGSGRRIIADTHLFWPNGLTIDYAGRRMYWVDAKHHVIERANLDGSHRKAVISQGLPHPFAITVFEDSLYWTDWHTKSINSANKFTGKNQEIIRNKLHFPMDIHTLHPQRQPAGKNRCGDNNGGCTHLCLPSGQNYTCACPTGFRKISSHACAQSLDKFLLFARRMDIRRISFDTEDLSDDVIPLADVRSAVALDWDSRDDHVYWTDVSTDTISRAKWDGTGQEVVVDTSLESPAGLAIDWVTNKLYWTDAGTDRIEVANTDGSMRTVLIWENLDRPRDIVVEPMGGYMYWTDWGASPKIERAGMDASGRQVIISSNLTWPNGLAIDYGSQRLYWADAGMKTIEFAGLDGSKRKVLIGSQLPHPFGLTLYGERIYWTDWQTKSIQSADRLTGLDRETLQENLENLMDIHVFHRRRPPVSTPCAMENGGCSHLCLRSPNPSGFSCTCPTGINLLSDGKTCSPGMNSFLIFARRIDIRMVSLDIPYFADVVVPINITMKNTIAIGVDPQEGKVYWSDSTLHRISRANLDGSQHEDIITTGLQTTDGLAVDAIGRKVYWTDTGTNRIEVGNLDGSMRKVLVWQNLDSPRAIVLYHEMGFMYWTDWGENAKLERSGMDGSDRAVLINNNLGWPNGLTVDKASSQLLWADAHTERIEAADLNGANRHTLVSPVQHPYGLTLLDSYIYWTDWQTRSIHRADKGTGSNVILVRSNLPGLMDMQAVDRAQPLGFNKCGSRNGGCSHLCLPRPSGFSCACPTGIQLKGDGKTCDPSPETYLLFSSRGSIRRISLDTSDHTDVHVPVPELNNVISLDYDSVDGKVYYTDVFLDVIRRADLNGSNMETVIGRGLKTTDGLAVDWVARNLYWTDTGRNTIEASRLDGSCRKVLINNSLDEPRAIAVFPRKGYLFWTDWGHIAKIERANLDGSERKVLINTDLGWPNGLTLDYDTRRIYWVDAHLDRIESADLNGKLRQVLVSHVSHPFALTQQDRWIYWTDWQTKSIQRVDKYSGRNKETVLANVEGLMDIIVVSPQRQTGTNACGVNNGGCTHLCFARASDFVCACPDEPDSRPCSLVPGLVPPAPRATGMSEKSPVLPNTPPTTLYSSTTRTRTSLEEVEGRCSERDARLGLCARSNDAVPAAPGEGLHISYAIGGLLSILLILVVIAALMLYRHKKSKFTDPGMGNLTYSNPSYRTSTQEVKIEAIPKPAMYNQLCYKKEGGPDHNYTKEKIKIVEGICLLSGDDAEWDDLKQLRSSRGGLLRDHVCMKTDTVSIQASSGSLDDTETEQLLQEEQSECSSVHTAATPERRGSLPDTGWKHERKLSSESQV

Gene Information

Entrez Gene ID
4038
Gene Name
low density lipoprotein receptor-related protein 4
Gene Symbol
LRP4
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0009986 IDA:UniProt C cell surface
GO:0030425 ISS:UniProt C dendrite
GO:0016021 IEA:UniProtKB-KW C integral component of membrane
GO:0031594 ISS:UniProt C neuromuscular junction
GO:0043025 ISS:UniProt C neuronal cell body
GO:0014069 ISS:UniProt C postsynaptic density
GO:0097060 ISS:UniProt C synaptic membrane
GO:0005509 IEA:InterPro F calcium ion binding
GO:0030971 ISS:UniProt F receptor tyrosine kinase binding
GO:0097110 ISS:UniProt F scaffold protein binding
GO:0016055 IEA:UniProtKB-KW P Wnt signaling pathway
GO:0048813 ISS:UniProt P dendrite morphogenesis
GO:0009953 IEA:Ensembl P dorsal/ventral pattern formation
GO:0042733 IEA:Ensembl P embryonic digit morphogenesis
GO:0006897 IEA:UniProtKB-KW P endocytosis
GO:0030198 TAS:Reactome P extracellular matrix organization
GO:0001942 IEA:Ensembl P hair follicle development
GO:0001822 IDA:UniProt P kidney development
GO:0060173 IDA:UniProt P limb development
GO:0050771 ISS:UniProt P negative regulation of axonogenesis
GO:0090090 IMP:UniProtKB P negative regulation of canonical Wnt signaling pathway
GO:0030279 IMP:UniProtKB P negative regulation of ossification
GO:0042475 IEA:Ensembl P odontogenesis of dentin-containing tooth
GO:1901631 ISS:UniProt P positive regulation of presynaptic membrane organization
GO:0097104 ISS:UniProt P postsynaptic membrane assembly
GO:0097105 ISS:UniProt P presynaptic membrane assembly
GO:0051290 ISS:UniProt P protein heterotetramerization
GO:0009954 IEA:Ensembl P proximal/distal pattern formation
GO:0001932 IEA:Ensembl P regulation of protein phosphorylation
GO:0071340 ISS:UniProt P skeletal muscle acetylcholine-gated channel clustering
GO:0050808 ISS:UniProt P synapse organization
GO:0051124 ISS:UniProt P synaptic growth at neuromuscular junction

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_163906 ECM proteoglycans
REACT_118779 Extracellular matrix organization

Domain Information

InterPro Annotations

Accession Description
IPR026823 Complement Clr-like EGF domain
IPR001881 EGF-like calcium-binding domain
IPR018097 EGF-like calcium-binding, conserved site
IPR013032 EGF-like, conserved site
IPR000152 EGF-type aspartate/asparagine hydroxylation site
IPR000742 Epidermal growth factor-like domain
IPR009030 Insulin-like growth factor binding protein, N-terminal
IPR000033 LDLR class B repeat
IPR002172 Low-density lipoprotein (LDL) receptor class A repeat
IPR023415 Low-density lipoprotein (LDL) receptor class A, conserved site
IPR011042 Six-bladed beta-propeller, TolB-like

UniProt Annotations

Entry Information

Gene Name
low density lipoprotein receptor-related protein 4
Protein Entry
LRP4_HUMAN
UniProt ID
O75096
Species
Human

Comments

Comment Type Description
Disease Cenani-Lenz syndactyly syndrome (CLSS) [MIM
Disease Sclerosteosis 2 (SOST2) [MIM
Function Mediates SOST-dependent inhibition of bone formation. Functions as a specific facilitator of SOST-mediated inhibition of Wnt signaling. Plays a key role in the formation and the maintenance of the neuromuscular junction (NMJ), the synapse between motor neuron and skeletal muscle. Directly binds AGRIN and recruits it to the MUSK signaling complex. Mediates the AGRIN- induced phosphorylation of MUSK, the kinase of the complex. The activation of MUSK in myotubes induces the formation of NMJ by regulating different processes including the transcription of specific genes and the clustering of AChR in the postsynaptic membrane. Alternatively, may be involved in the negative regulation of the canonical Wnt signaling pathway, being able to antagonize the LRP6-mediated activation of this pathway. More generally, has been proposed to function as a cell surface endocytic receptor binding and internalizing extracellular ligands for degradation by lysosomes. {ECO
Sequence Caution Sequence=BAE19679.1; Type=Erroneous initiation; Evidence= ;
Similarity Belongs to the LDLR family.
Similarity Contains 20 LDL-receptor class B repeats.
Similarity Contains 3 EGF-like domains.
Similarity Contains 8 LDL-receptor class A domains.
Subcellular Location Membrane ; Single-pass type I membrane protein .
Subunit Homooligomer. Interacts with MUSK; the heterodimer forms an AGRIN receptor complex that binds AGRIN resulting in activation of MUSK (By similarity). Interacts (via the extracellular domain) with SOST; the interaction facilitates the inhibition of Wnt signaling.
Tissue Specificity Expressed in bone; present in osteoblasts and osteocytes. No expression is observed in osteoclast. Expressed in several regions of the brain.

Identical and Related Proteins

Unique RefSeq proteins for LMP006691 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
157384998 RefSeq NP_002325 1905 low-density lipoprotein receptor-related protein 4 precursor

Identical Sequences to LMP006691 proteins

Reference Database Accession Length Protein Name
GI:157384998 SwissProt O75096.4 1905 RecName: Full=Low-density lipoprotein receptor-related protein 4; Short=LRP-4; AltName: Full=Multiple epidermal growth factor-like domains 7; Flags: Precursor [Homo sapiens]

Related Sequences to LMP006691 proteins

Reference Database Accession Length Protein Name
GI:157384998 DBBJ BAD83615.1 1905 low density lipoprotein receptor-related protein 10 [Homo sapiens]
GI:157384998 GenBank AAI36669.1 1905 Low density lipoprotein receptor-related protein 4 [Homo sapiens]
GI:157384998 GenBank AAI36668.1 1905 Low density lipoprotein receptor-related protein 4 [Homo sapiens]
GI:157384998 GenBank JAA10212.1 1905 low density lipoprotein receptor-related protein 4 [Pan troglodytes]
GI:157384998 GenBank AHD73845.1 1905 Sequence 12303 from patent US 8586006
GI:157384998 RefSeq XP_009458674.1 1979 PREDICTED: low-density lipoprotein receptor-related protein 4, partial [Pan troglodytes]