Ms X Fibrillin-1

Species Reactivity	Key Applications	Host	Format	Antibody Type
H	ELISA, IP, WB, ICC	M	Purified	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

ELISA, IP, WB, ICC

Purified

Monoclonal Antibody

Description
Catalogue Number	MAB2502
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-Fibrillin-1 Antibody, NT, clone 26

Description

Catalogue Number

MAB2502

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-Fibrillin-1 Antibody, NT, clone 26

Product Information
Format	Purified
Presentation	Liquid at 1 mg/mL in 20 mM phosphate buffer, 250 mM NaCl, pH 7.6, containing 0.1% sodium azide.Note: Sodium azide is toxic. MSDS available upon request.

Product Information

Format

Purified

Presentation

Liquid at 1 mg/mL in 20 mM phosphate buffer, 250 mM NaCl, pH 7.6, containing 0.1% sodium azide.Note: Sodium azide is toxic. MSDS available upon request.

Applications
Application	Anti-Fibrillin-1 Antibody, N-terminus, clone 26 detects level of Fibrillin-1 & has been published & validated for use in ELISA, IP, WB & IC.
Key Applications	ELISA Immunoprecipitation Western Blotting Immunocytochemistry
Application Notes	Immunoblotting Immunofluorescence Immunoprecipitation ELISA Optimal working dilutions must be determined by end user.

Applications

Application

Anti-Fibrillin-1 Antibody, N-terminus, clone 26 detects level of Fibrillin-1 & has been published & validated for use in ELISA, IP, WB & IC.

Key Applications

ELISA
Immunoprecipitation
Western Blotting
Immunocytochemistry

Application Notes

Immunoblotting

Immunofluorescence

Immunoprecipitation

ELISA

Optimal working dilutions must be determined by end user.

Biological Information
Immunogen	Human Fibrillin-1
Epitope	N-terminus
Clone	26
Host	Mouse
Specificity	Monoclonal antibody MAB2502 recognizes human Fibrillin-1. Epitope mapping studies identify the binding site of this antibody to amino-terminal end of the molecule, between amino acid residues 45 and 450. The antibody is reactive with human, chicken, and bovine Fibrillin-1.
Isotype	IgG1
Species Reactivity	Human
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_000138.3
Entrez Gene Summary	This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome.
Gene Symbol	FBN1 FBN MASS WMS MFS1 OCTD SGS
UniProt Number	P35555
UniProt Summary	FUNCTION: SwissProt: P35555 # 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-1- containing microfibrils provide long-term force bearing structural support. SIZE: 2871 amino acids; 312312 Da SUBUNIT: Interacts with COL16A1. SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix. PTM: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils. DISEASE: SwissProt: P35555 # Defects in FBN1 are a cause of Marfan syndrome (MFS) [MIM:154700]. MFS is an autosomal dominant disorder that affects the skeletal, ocular, and cardiovascular systems. A wide variety of skeletal abnormalities occurs with MFS, including scoliosis, chest wall deformity, tall stature, abnormal joint mobility. Ectopia lentis occurs in up to about 80% of MFS patients and is almost always bilateral. The leading cause of premature death in MFS patients is progressive dilation of the aortic root and ascending aorta, causing aortic incompetence and dissection. The majority of the more than 600 mutations in FBN1 currently known are point mutations, the rest are frameshifts and splice site mutations. Marfan syndrome has been suggested in at least 2 historical figures, Abraham Lincoln and Paganini. & Defects in FBN1 are a cause of isolated ectopia lentis (EL) [MIM:129600]. The symptoms of this autosomal dominant fibrillinopathy overlap with those of Marfan syndrome, with the exclusion of the skeletal and cardiovascular manifestations. & Defects in FBN1 are the cause of autosomal dominant Weill-Marchesani syndrome (WMS) [MIM:608328]. WMS is a rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma. & Defects in FBN1 are a cause of Shprintzen-Goldberg craniosynostosis syndrome (SGS) [MIM:182212]. SGS is one of a group of disorders characterized by typical features of the Marfan syndrome along with premature closure of the sutures of the skull, causing deformities such as oxycephaly and scaphocephaly. & Defects in FBN1 are a cause of MASS syndrome [MIM:604308]. MASS syndrome is a heritable disorder of connective tissue characterized by involvement of the mitral valve, aorta, skeleton, and skin. MASS syndrome is closely resembling both the Marfan syndrome and the Barlow syndrome. However, no dislocation of the lenses or aneurysmal changes occur in the aorta, and the mitral valve prolapse is by no means invariable. SIMILARITY: SwissProt: P35555 ## Belongs to the fibrillin family. & Contains 47 EGF-like domains. & Contains 7 TGFBP (TGF-beta binding protein) repeats.

Biological Information

Immunogen

Human Fibrillin-1

Epitope

N-terminus

Clone

Host

Mouse

Specificity

Monoclonal antibody MAB2502 recognizes human Fibrillin-1. Epitope mapping studies identify the binding site of this antibody to amino-terminal end of the molecule, between amino acid residues 45 and 450. The antibody is reactive with human, chicken, and bovine Fibrillin-1.

Isotype

IgG1

Species Reactivity

Human

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NM_000138.3

Entrez Gene Summary

This gene encodes a member of the fibrillin family. The encoded protein is a large, extracellular matrix glycoprotein that serve as a structural component of 10-12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations in this gene are associated with Marfan syndrome, isolated ectopia lentis, autosomal dominant Weill-Marchesani syndrome, MASS syndrome, and Shprintzen-Goldberg craniosynostosis syndrome.

Gene Symbol

FBN1
FBN
MASS
WMS
MFS1
OCTD
SGS

UniProt Number

P35555

UniProt Summary

FUNCTION: SwissProt: P35555 # 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-1- containing microfibrils provide long-term force bearing structural support.
SIZE: 2871 amino acids; 312312 Da
SUBUNIT: Interacts with COL16A1.
SUBCELLULAR LOCATION: Secreted, extracellular space, extracellular matrix.
PTM: Forms intermolecular disulfide bonds either with other fibrillin-1 molecules or with other components of the microfibrils.
DISEASE: SwissProt: P35555 # Defects in FBN1 are a cause of Marfan syndrome (MFS) [MIM:154700]. MFS is an autosomal dominant disorder that affects the skeletal, ocular, and cardiovascular systems. A wide variety of skeletal abnormalities occurs with MFS, including scoliosis, chest wall deformity, tall stature, abnormal joint mobility. Ectopia lentis occurs in up to about 80% of MFS patients and is almost always bilateral. The leading cause of premature death in MFS patients is progressive dilation of the aortic root and ascending aorta, causing aortic incompetence and dissection. The majority of the more than 600 mutations in FBN1 currently known are point mutations, the rest are frameshifts and splice site mutations. Marfan syndrome has been suggested in at least 2 historical figures, Abraham Lincoln and Paganini. & Defects in FBN1 are a cause of isolated ectopia lentis (EL) [MIM:129600]. The symptoms of this autosomal dominant fibrillinopathy overlap with those of Marfan syndrome, with the exclusion of the skeletal and cardiovascular manifestations. & Defects in FBN1 are the cause of autosomal dominant Weill-Marchesani syndrome (WMS) [MIM:608328]. WMS is a rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma. & Defects in FBN1 are a cause of Shprintzen-Goldberg craniosynostosis syndrome (SGS) [MIM:182212]. SGS is one of a group of disorders characterized by typical features of the Marfan syndrome along with premature closure of the sutures of the skull, causing deformities such as oxycephaly and scaphocephaly. & Defects in FBN1 are a cause of MASS syndrome [MIM:604308]. MASS syndrome is a heritable disorder of connective tissue characterized by involvement of the mitral valve, aorta, skeleton, and skin. MASS syndrome is closely resembling both the Marfan syndrome and the Barlow syndrome. However, no dislocation of the lenses or aneurysmal changes occur in the aorta, and the mitral valve prolapse is by no means invariable.
SIMILARITY: SwissProt: P35555 ## Belongs to the fibrillin family. & Contains 47 EGF-like domains. & Contains 7 TGFBP (TGF-beta binding protein) repeats.

Product Usage Statements
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	Maintain refrigerated at 2-8°C in undiluted aliquots for up to 12 months.

Storage and Shipping Information

Storage Conditions

Maintain refrigerated at 2-8°C in undiluted aliquots for up to 12 months.

Packaging Information
Material Size	100 µg

Packaging Information

Material Size

100 µg

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

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