Name: Anti-LRP4 Antibody (CAB10172)
Brand: Developmental Biology
SKU: CAB10172
Availability: OutOfStock

Antibody Name:	Anti-LRP4 Antibody
Antibody SKU:	CAB10172
Antibody Size:	20uL, 50uL, 100uL
Application:	WB
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1500-1725 of human LRP4 (NP_002325.2).

Application:	WB
Recommended Dilution:	WB 1:500 - 1:2000
Reactivity:	Human, Mouse
Positive Samples:	SKOV3, HepG2, Mouse lung, Mouse liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1500-1725 of human LRP4 (NP_002325.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	NLDG SERK VLIN TDLG WPNG LTLD YDTR RIYW VDAH LDRI ESAD LNGK LRQV LVSH VSHP FALT QQDR WIYW TDWQ TKSI QRVD KYSG RNKE TVLA NVEG LMDI IVVS PQRQ TGTN ACGV NNGG CTHL CFAR ASDF VCAC PDEP DSRP CSLV PGLV PPAP RATG MSEK SPVL PNTP PTTL YSST TRTR TSLE EVEG RCSE RDAR LGLC ARSN DAVP AAPG EGLH IS
Gene ID:	4038
Uniprot:	O75096
Cellular Location:	Membrane, Single-pass type I membrane protein
Calculated MW:	212kDa
Observed MW:	200kDa

Synonyms:	LRP4, CLSS, CMS17, LRP-4, LRP10, MEGF7, SOST2
Background:	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.

UniProt Protein Function:	LRP4: 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. Defects in LRP4 are the cause of Cenani-Lenz syndactyly syndrome (CLSS). It is a congenital malformation syndrome defined as complete and complex syndactyly of the hands combined with malformations of the forearm bones and similar manifestations in the lower limbs. Defects in LRP4 are the cause of sclerosteosis type 2 (SOST2). A sclerosing bone dysplasia characterized by a generalized hyperostosis and sclerosis leading to a markedly thickened skull, with mandible, ribs, clavicles and all long bones also being affected. Due to narrowing of the foramina of the cranial nerves, facial nerve palsy, hearing loss and atrophy of the optic nerves can occur. Sclerosteosis is clinically and radiologically very similar to van Buchem disease, mainly differentiated by hand malformations and a large stature in sclerosteosis patients. Belongs to the LDLR family.
UniProt Protein Details:	Protein type:Receptor, misc.; Membrane protein, integral; Cell surface Chromosomal Location of Human Ortholog: 11p11.2 Cellular Component: cell surface; cell soma; dendrite; postsynaptic density; integral to membrane; flotillin complex; neuromuscular junction Molecular Function:protein binding; protein homodimerization activity; apolipoprotein binding; calcium ion binding; receptor tyrosine kinase binding Biological Process: limb development; extracellular matrix organization and biogenesis; regulation of protein amino acid phosphorylation; Wnt receptor signaling pathway; dendrite morphogenesis; endocytosis; odontogenesis of dentine-containing teeth; dorsal/ventral pattern formation; synaptic growth at neuromuscular junction; hair follicle development; negative regulation of ossification; synapse organization and biogenesis; protein heterotetramerization; negative regulation of axonogenesis; embryonic digit morphogenesis; kidney development; proximal/distal pattern formation Disease: Myasthenic Syndrome, Congenital, 17; Cenani-lenz Syndactyly Syndrome; Sclerosteosis 2
NCBI 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]
UniProt Code:	O75096
NCBI GenInfo Identifier:	157384998
NCBI Gene ID:	4038
NCBI Accession:	NP_002325.2
UniProt Secondary Accession:	O75096,Q4AC85, Q5KTZ5, B2RN39,
UniProt Related Accession:	O75096
Molecular Weight:	212,045 Da
NCBI Full Name:	low-density lipoprotein receptor-related protein 4
NCBI Synonym Full Names:	low density lipoprotein receptor-related protein 4
NCBI Official Symbol:	LRP4
NCBI Official Synonym Symbols:	CLSS; LRP-4; LRP10; MEGF7; SOST2
NCBI Protein Information:	low-density lipoprotein receptor-related protein 4; multiple epidermal growth factor-like domains 7
UniProt Protein Name:	Low-density lipoprotein receptor-related protein 4
UniProt Synonym Protein Names:	Multiple epidermal growth factor-like domains 7
Protein Family:	Low-density lipoprotein receptor-related protein
UniProt Gene Name:	LRP4
UniProt Entry Name:	LRP4_HUMAN

Western blot analysis of extracts of various cell lines, using LRP4 antibody (CAB10172) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST.

ELISA Kits

Human Low-density lipoprotein receptor-related protein 4 / LRP4 ELISA Kit

Human Low-density lipoprotein receptor-related protein 4 (LRP4) ELISA Kit

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)