Cell Biology Antibodies 12

Anti-FBN2 Antibody (CAB9414)

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SKU:
CAB9414
Product Type:
Antibody
Applications:
WB
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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Antibody Name:Anti-FBN2 Antibody
Antibody SKU:CAB9414
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 2733-2912 of human FBN2 (NP_001990.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:LO2, Mouse brain
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 2733-2912 of human FBN2 (NP_001990.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:CVSG MGFN KGQY LSLD TEVD EENA LSPE ACYE CKIN GYSK KDSR QKRS IHEP DPTA VEQI SLES VDMD SPVN MKFN LSHL GSKE HILE LRPA IQPL NNHI RYVI SQGN DDSV FRIH QRNG LSYL HTAK KKLM PGTY TLEI TSIP LYKK KELK KLEE SNED DYLL GELG EALR MRLQ IQLY
Gene ID:2201
Uniprot:P35556
Cellular Location:Secreted, extracellular matrix, extracellular space
Calculated MW:157kDa/314kDa
Observed MW:290kDa
Synonyms:FBN2, CCA, DA9, EOMD
Background:The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly.
UniProt Protein Function:FBN2: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2- containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects in FBN2 are the cause of distal arthrogryposis type 9 (DA9). A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. DA9 is a connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Belongs to the fibrillin family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 5q23-q31

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; microfibril; extracellular region

Molecular Function:protein binding; extracellular matrix structural constituent; calcium ion binding

Biological Process: positive regulation of osteoblast differentiation; extracellular matrix disassembly; extracellular matrix organization and biogenesis; anatomical structure morphogenesis; positive regulation of bone mineralization; embryonic limb morphogenesis

Disease: Macular Degeneration, Early-onset; Arthrogryposis, Distal, Type 9

NCBI Summary:The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq, Jul 2008]
UniProt Code:P35556
NCBI GenInfo Identifier:66346695
NCBI Gene ID:2201
NCBI Accession:NP_001990.2
UniProt Secondary Accession:P35556,Q59ES6, B4DU01,
UniProt Related Accession:P35556
Molecular Weight:157,690 Da
NCBI Full Name:fibrillin-2
NCBI Synonym Full Names:fibrillin 2
NCBI Official Symbol:FBN2  
NCBI Official Synonym Symbols:CCA; DA9  
NCBI Protein Information:fibrillin-2; fibrillin 5
UniProt Protein Name:Fibrillin-2
Protein Family:Fibrillin
UniProt Gene Name:FBN2  
UniProt Entry Name:FBN2_HUMAN
Anti-FBN2 Polyclonal Antibody (CAB9414)Western blot analysis of extracts of various cell lines, using FBN2 antibody (CAB9414) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
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