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Fibrillin 2 Polyclonal Antibody

Applications

  • ELISA
  • IHC-P
  • IHC-F
  • IF(IHC-P)
  • IF(IHC-F)
  • IF(ICC)

Predicted Reactivity

  • Human
  • Mouse
  • Rat
  • Dog
  • Cow
  • Sheep
  • Pig
  • Horse
  • Chicken
  • Rabbit
Overview
Catalog # bs-12166R
Product Name Fibrillin 2 Polyclonal Antibody
Applications ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC)
Predicted Reactivity Human, Mouse, Rat, Dog, Cow, Sheep, Pig, Horse, Chicken, Rabbit
Specifications
Conjugation Unconjugated
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Fibrillin 2
Immunogen Range 1001-1200/2912
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage Condition Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
Target
Subcellular location Secreted
Synonyms CCA; congenital contractural arachnodactyly Marfanoid-like; DA9; FBN2; FBN2_HUMAN; fibrillin 2 congenital contractural arachnodactyly; Fibrillin-2.
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.
Application Dilution
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200