Name: Anti-ANTXR2 Antibody (CAB6526)
Brand: Cell Biology Antibodies 10
SKU: CAB6526
Availability: OutOfStock

Antibody Name:	Anti-ANTXR2 Antibody
Antibody SKU:	CAB6526
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 339-488 of human ANTXR2 (NP_477520.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	Mouse eye, Rat lung

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 339-488 of human ANTXR2 (NP_477520.2).
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:	WFWP LCCK VVIK DPPP PPAP APKE EEEE PLPT KKWP TVDA SYYG GRGV GGIK RMEV RWGD KGST EEGA RLEK AKNA VVKI PEET EEPI RPRP PRPK PTHQ PPQT KWYT PIKG RLDA LWAL LRRQ YDRV SLMR PQEG DEGR CINF SRVP SQ
Gene ID:	118429
Uniprot:	P58335
Cellular Location:	Cell membrane, Endoplasmic reticulum membrane, Secreted, Single-pass type I membrane protein, Single-pass type I membrane protein
Calculated MW:	34kDa/42kDa/53kDa
Observed MW:	54kDa

Synonyms:	ANTXR2, CMG-2, CMG2, HFS, ISH, JHF
Background:	This gene encodes a receptor for anthrax toxin. The protein binds to collagen IV and laminin, suggesting that it may be involved in extracellular matrix adhesion. Mutations in this gene cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. Multiple transcript variants encoding different isoforms have been found for this gene.

UniProt Protein Function:	ANTXR2: Necessary for cellular interactions with laminin and the extracellular matrix. Defects in ANTXR2 are the cause of infantile systemic hyalinosis (ISH). This autosomal recessive syndrome is similar to JHF, but has an earlier onset and a more severe course. Symptoms appear at birth or within the first months of life, with painful, swollen joint contractures, osteopenia, osteoporosis and livid red hyperpigmentation over bony prominences. Patients develop multiple subcutaneous skin tumors and gingival hypertrophy. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to death within the first 2 years of life. Surviving children may suffer from severely reduced mobility due to joint contractures. Defects in ANTXR2 are the cause of juvenile hyaline fibromatosis (JHF). JHF is an autosomal recessive syndrome that is similar to ISH but takes a milder course. It is characterized by hyaline deposition in the dermis, multiple subcutaneous skin tumors and gingival hypertrophy, followed by progressive joint contractions, osteopenia and osteoporosis that may lead to a severe limitation of mobility. Belongs to the ATR family. 4 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Membrane protein, integral; Receptor, misc. Chromosomal Location of Human Ortholog: 4q21.21 Cellular Component: endoplasmic reticulum membrane; extracellular region; plasma membrane; integral to membrane; endosome membrane; external side of plasma membrane Molecular Function:protein binding; metal ion binding; receptor activity Biological Process: reproductive process; pathogenesis Disease: Hyaline Fibromatosis Syndrome
NCBI Summary:	This gene encodes a receptor for anthrax toxin. The protein binds to collagen IV and laminin, suggesting that it may be involved in extracellular matrix adhesion. Mutations in this gene cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]
UniProt Code:	P58335
NCBI GenInfo Identifier:	306526289
NCBI Gene ID:	118429
NCBI Accession:	P58335.5
UniProt Secondary Accession:	P58335,Q4W5H6, Q59E98, Q5JPE9, Q86UI1, Q8N4J8, Q8NB13 Q96NC7,
UniProt Related Accession:	P58335
Molecular Weight:	53,493 Da
NCBI Full Name:	Anthrax toxin receptor 2
NCBI Synonym Full Names:	anthrax toxin receptor 2
NCBI Official Symbol:	ANTXR2
NCBI Official Synonym Symbols:	HFS; ISH; JHF; CMG2; CMG-2
NCBI Protein Information:	anthrax toxin receptor 2; capillary morphogenesis protein 2; capillary morphogenesis gene 2 protein
UniProt Protein Name:	Anthrax toxin receptor 2
UniProt Synonym Protein Names:	Capillary morphogenesis gene 2 protein; CMG-2
Protein Family:	Anthrax toxin receptor
UniProt Gene Name:	ANTXR2
UniProt Entry Name:	ANTR2_HUMAN

	Western blot analysis of extracts of various cell lines, using ANTXR2 Rabbit pAb (CAB6526) 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. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
	Immunohistochemistry of paraffin-embedded human stomach using ANTXR2 antibody (CAB6526) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse kidney using ANTXR2 antibody (CAB6526) at dilution of 1:100 (40x lens).