Human ANTXR2 Recombinant Protein (RPPB2745)

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RPPB2745

Description

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Product Name:Human ANTXR2 Recombinant Protein
Product Code:RPPB2745
Size:10µg
Species:Human
Target:ANTXR2
Synonyms:Anthrax toxin receptor 2, ANTXR2, Capillary morphogenesis gene 2 protein, CMG-2, CMG2, m CMG2, HFS, ISH, JHF.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The ANTXR2 solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 10% glycerol, 1mM DTT and 0.1M NaCl.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSQEQPSCR RAFDLYFVLD KSGSVANNWI EIYNFVQQLA ERFVSPEMRL SFIVFSSQAT IILPLTGDRG KISKGLEDLK RVSPVGETYI HEGLKLANEQ IQKAGGLKTS SIIIALTDGK LDGLVPSYAE KEAKISRSLG ASVYCVGVLD FEQAQLERIA DSKEQVFPVK GGFQALKGII NSILAQSCTE ILELQPSSVC VGEEFQIVLS GRGFMLGSRN GSVLCTYTVN ETYTTSVKPV SVQLNSMLCP APILNKAGET LDVSVSFNGG KSVISGSLIV TATECSN

Anthrax toxin receptor 2, also known as ANTXR2, takes part in the configuration of little blood vessels (capillaries). ANTXR2 helps the toxin that leads to anthrax to attach to cells and trigger illness. ANTXR2 is necessary for cellular interactions with laminin and the extracellular matrix.

ANTXR2 Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 307 amino acids (34-317) and having a molecular mass of 33 kDa.ANTXR2 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

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
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Additional Information

Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P58335
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