Fibrilline 2 Polyklonale Antilichaam, FITC Vervoeging

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.

CCA; congenital contractural arachnodactyly Marfanoid-like; DA9; FBN2; FBN2_HUMAN; fibrillin 2 congenital contractural arachnodactyly; Fibrillin-2.

Secreted

Rat

1001-1200/2912

Fibrillin 2

FITC

KLH conjugated synthetic peptide derived from human Fibrillin 2

IF (IHC-P), IF (IHC-F), IF (ICC)

Purified by Protein A.

494nm/518nm

1µg/µl

IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Unmodified

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Human, Mouse, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit