YRP12171-01 [Polyclonal Antibody]
Fibrillin 2 Rabbit Polyclonal Antibody
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Species:   Rabbit
Applications:   IHC IF
Immunogen Range:   KLH conjugated synthetic peptide derived from human Fibrillin 2
Clonality:   Polyclonal Antibody
Isotype:   IgG
GENE ID:  
Swiss Prot:  
Synonyms:   CCA, congenital contractural arachnodactyly Marfanoid-like, DA9, FBN2, FBN2_HUMAN, fibrillin 2 congenital contractural arachnodactyly, Fibrillin-2.
Purification:   Purified by Protein A.
Storage:   Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months
Background:   Extracellular glycoproteins fibrillin-1 and -2 are major components of connective tissue microfibrils. Fibrillin-2 containing microfibrils regulate the early process of elastic fiber assembly in tissue. Mutations in the fibrillin-2 gene resulting in impaired assembly of fibrillin-2 may lead to molecular congenital contractural arachnodactyly. Fibrillin-2 constitutes the backbone of microfibrils which insert directly into the lamina densa of basement membranes. Epithelial cells primarily deposit fibrillin into the extracellular matrix in a nonfibrillar form. Mutations in the 8-cysteine motif of Fibrillin-2 alters its binding to microfibril-associated glycoprotein-1 (MAGP-1), which may increase the severity of congenital contractural arachnodactyly.
Caculated MW:   /
Observed MW:   Refer to Figures
Applications:   IHC 1:100-1:500
IF 1:50-1:200
Reacitivity:   Human, Mouse, Rat
For research use only. Not intended for diagnostic or therapeutic use!
Additional information