Anti-ANTXR2 antibody (AA37203)

$187.00


Alternative names Anthrax toxin receptor 2, Capillary morphogenesis gene 2 protein, CMG-2, ANTXR2, CMG2, ANTR2_HUMAN, P58335, 118429
Source 489?488?
Species Reactivity Human
Applications IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ANTXR2 antibody (AA37203)
Catalog number AA37203
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Anthrax toxin receptor 2 antibody, Capillary morphogenesis gene 2 protein antibody, CMG-2 antibody, ANTXR2 antibody, CMG2 antibody, ANTR2_HUMAN antibody


Biological Information

Concentration 1 mg/ml
Isotype IgG
Purification method Purified by affinity chromatography


Target

Target Name ANTXR2
SwissProt ID Link P58335
Function Necessary for cellular interactions with laminin and the extracellular matrix.
Subcellular Location Isoform 1: Cell membrane; Single-pass type I membrane protein. Expressed at the cell surface.; Isoform 2: Endoplasmic reticulum membrane; Single-pass type I membrane protein. Expressed predominantly within the endoplasmic reticulum and not at the plasma membrane.; Isoform 3: Secreted.
Involvement in Disease Hyaline fibromatosis syndrome: 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. The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Expressed in prostate, thymus, ovary, testis, pancreas, colon, heart, kidney, lung, liver, peripheral blood leukocytes, placenta, skeletal muscle, small intestine and spleen.
Subunit Structure Binds laminin, and possibly also collagen type IV. Binds to the protective antigen (PA) of Bacillus anthracis in a divalent cation-dependent manner, with the following preference: calcium > manganese > magnesium > zinc. Binding of PA leads to heptamerization of the receptor-PA complex.
Sequence Similarities Belongs to the ATR family. Contains 1 VWFA domain.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 C. Avoid multiple freeze/thaw cycles.