Rabbit ANTXR2 Polyclonal Antibody | anti-ANTXR2 antibody

ANTXR2 Rabbit Polyclonal

Cat. Num. AAA766683

• Gene Names: ANTXR2; HFS; ISH; JHF; CMG2; CMG-2
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Immunohistochemistry, Western Blot, Immunoprecipitation
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: ANTXR2; Polyclonal Antibody; ANTXR2 Rabbit Polyclonal; anti-ANTXR2 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG1
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 411

• Applicable Applications for anti-ANTXR2 antibody: ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB), Immunoprecipitation (IP)
• Immunogen: Anthrax toxin receptor 2
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human stomach using MBS766683 (ANTXR2 antibody) at dilution of 1:100)

Western Blot (WB)

(HEK-293 cells were subjected to SDS PAGE followed by western blot with MBS766683 (ANTXR2 Antibody) at dilution of 1:600)

NCBI and Uniprot Product Information

• NCBI GI #: 557948116
• NCBI GeneID: 118429
• NCBI Accession #: NP_001273710.1
• NCBI GenBank Nucleotide #: NM_001286781.1
• Molecular Weight: 53,493 Da
• NCBI Official Full Name: anthrax toxin receptor 2 isoform 3
• NCBI Official 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
• UniProt Protein Name: Anthrax toxin receptor 2
• Protein Family: Anthrax toxin receptor
• UniProt Gene Name: ANTXR2
• UniProt Synonym Gene Names: CMG2; CMG-2
• UniProt Entry Name: ANTR2_HUMAN

NCBI Description

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 Description

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.

Protein type: Membrane protein, integral; Receptor, misc.

Chromosomal Location of Human Ortholog: 4q21.21

Cellular Component: endoplasmic reticulum membrane; endosome membrane; external side of plasma membrane; extracellular region; integral to membrane; plasma membrane

Molecular Function: metal ion binding; protein binding; receptor activity

Biological Process: reproductive process

Disease: Hyaline Fibromatosis Syndrome