Mouse Anti-ANTXR2 Recombinant Antibody (CBYC-A621) (CBMAB-A2869-YC)

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Basic Information

Host Animal
Mouse
Clone
CBYC-A621
Application
ELISA
Immunogen
Mouse myeloma cell line NS0-derived recombinant human CMG-2/ANTXR2 Ser22-Arg200.
Host Species
Mouse
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Lyophilized
Buffer
PBS, Trehalose
Preservative
None
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
More Infomation

Target

Full Name
Anthrax Toxin Receptor 2
Introduction
ANTXR2 is 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.
Entrez Gene ID
Human118429
Mouse71914
Rat305633
UniProt ID
HumanP58335
MouseQ6DFX2
RatF1LT78
Alternative Names
Anthrax Toxin Receptor 2; Capillary Morphogenesis Gene 2 Protein; Capillary Morphogenesis Protein 2; CMG-2; CMG2; HFS; ISH; JHF;
Function
Necessary for cellular interactions with laminin and the extracellular matrix.
Biological Process
Toxin transport Source: GO_Central
Cellular Location
Isoform 1: Cell membrane. Expressed at the cell surface.
Isoform 2: Endoplasmic reticulum membrane. Expressed predominantly within the endoplasmic reticulum and not at the plasma membrane.
Isoform 3: Secreted
Involvement in disease
Hyaline fibromatosis syndrome (HFS): An autosomal recessive syndrome characterized by abnormal growth of hyalinized fibrous tissue usually affecting subcutaneous regions on the scalp, ears, neck, face, hands, and feet. The lesions appear as pearly papules or fleshy nodules. Additional features include gingival hypertrophy, progressive joint contractures resulting in severe limitation of mobility, osteopenia, and osteoporosis. Disease severity is variable. Some individuals manifest symptoms in infancy and have additional visceral or systemic involvement. Hyaline deposits in multiple organs, recurrent infections and intractable diarrhea often lead to early death. Surviving children may suffer from severely reduced mobility due to joint contractures. Other patients have later onset of a milder disorder affecting only the face and digits.
Topology
Extracellular: 34-318 aa
Helical: 319-341 aa
Cytoplasmic: 342-489 aa
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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