Anti-Fibrillin 2 antibody
英文名称 | Fibrillin 2 |
中文名称 | 原纤维蛋白2抗体 |
别 名 | CCA; congenital contractural arachnodactyly (Marfanoid-like); DA9; FBN2; FBN2_HUMAN; fibrillin 2 (congenital contractural arachnodactyly); Fibrillin-2. |
DATASHEET
Host:Rabbit
Target Protein:Fibrillin 2
IR:Immunogen Range:1001-1200/2912
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:2201
Swiss Prot:P35556
Source:KLH conjugated synthetic peptide derived from human Fibrillin 2:1001-1200/2912
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
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.
Size:100ul
Concentration:1mg/ml
Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Chicken
Dog
Pig
Cow
Horse
Rabbit
Sheep
.
For research use only. Not intended for diagnostic or therapeutic use.
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