Anti-CD45 (human), FITC, clone HI30

Species Reactivity	Key Applications	Host	Format	Antibody Type
H, M, R	ELISA, ICC, IF, WB	M	Ascites	Monoclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

H, M, R

ELISA, ICC, IF, WB

Ascites

Monoclonal Antibody

Description
Catalogue Number	MAB1594
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-Frataxin Antibody, exon 4, clone 1G2
Alternate Names	Friedreich ataxia Friedreich ataxia protein
Background Information	Frataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle (Campuzano, 1996; Koutnikova, 1997). Frataxin is expressed as a 30 kDa precursor (transient; 210 amino acids) that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase (MPP) to yield the mature protein (Koutnikova, 1998). The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20 kDa (aa 42-210). Further cleavage of the N-terminus of this 20 kDa intermediate by MPP results in the mature 18 kDa frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich's ataxia, an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy. In the majority of cases of Friedreich's ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription (Campuzano, 1997; Bidichandani, 1998). This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress (Foury, 1997; for review see Puccio, 2000).

Description

Catalogue Number

MAB1594

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-Frataxin Antibody, exon 4, clone 1G2

Alternate Names

Friedreich ataxia
Friedreich ataxia protein

Background Information

Frataxin is a monomeric mitochondrial protein that is believed to be involved in iron homeostasis through an unknown mechanism. Expression of frataxin is highest in tissue rich with mitochondria including liver, heart, and skeletal muscle (Campuzano, 1996; Koutnikova, 1997). Frataxin is expressed as a 30 kDa precursor (transient; 210 amino acids) that is processed within in the mitochondria in two steps catalysed by the mitochondrial processing peptidase (MPP) to yield the mature protein (Koutnikova, 1998). The first step involves cleavage of the first 41 N-terminal amino acids by MPP yielding a transient intermediate of approximately 20 kDa (aa 42-210). Further cleavage of the N-terminus of this 20 kDa intermediate by MPP results in the mature 18 kDa frataxin protein (aa 56-210). Defects in the gene encoding frataxin are implicated as the cause of Friedreich's ataxia, an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy. In the majority of cases of Friedreich's ataxia, there is an expansion of a trinucleotide repeat in the first intron of the gene encoding frataxin resulting in a marked decrease in frataxin expression, perhaps due to the formation of some unusual yet stable DNA structure that interferes with transcription (Campuzano, 1997; Bidichandani, 1998). This reduction in frataxin expression results in the accumulation of unchelated iron in the mitochondria, inhibition of mitochondrial iron-sulfer proteins, and iron mediated oxidative stress (Foury, 1997; for review see Puccio, 2000).

Product Information
Format	Ascites
Control	Liver, heart or skeletal muscle.
Presentation	Mouse monoclonal ascites IgG1κ in buffer containing liquid with no preservatives.

Product Information

Format

Ascites

Control

Liver, heart or skeletal muscle.

Presentation

Mouse monoclonal ascites IgG1κ in buffer containing liquid with no preservatives.

Applications
Application	Detect Frataxin using this Anti-Frataxin Antibody, exon 4, clone 1G2 validated for use in ELISA, IC, IF & WB.
Key Applications	ELISA Immunocytochemistry Immunofluorescence Western Blotting
Application Notes	Immunofluorescence: 1:100-1:1,000. Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to the subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody. ELISA: A previous lot of this antibody was used on ELISA. Western blot (natural and recombinant protein): 1:5,000; mitochondrial preparations are recommended for consist signals (see Santos, 2001). Optimal working dilutions must be determined by the end user.

Applications

Application

Detect Frataxin using this Anti-Frataxin Antibody, exon 4, clone 1G2 validated for use in ELISA, IC, IF & WB.

Key Applications

ELISA
Immunocytochemistry
Immunofluorescence
Western Blotting

Application Notes

Immunofluorescence:
1:100-1:1,000. Fixation of cells in ice cold acetone or 4% paraformaldehyde is recommended. Due to the subcellular localization of frataxin in the mitochondria, cells should be permeabilized in the presence of detergent prior to incubation with primary antibody.

ELISA:
A previous lot of this antibody was used on ELISA.

Western blot (natural and recombinant protein):
1:5,000; mitochondrial preparations are recommended for consist signals (see Santos, 2001).

Optimal working dilutions must be determined by the end user.

Biological Information
Immunogen	Full length human Frataxin fused to TrpE.
Epitope	exon 4
Clone	1G2
Concentration	Please refer to the Certificate of Analysis for the lot-specific concentration.
Host	Mouse
Specificity	Human Frataxin. MAB1594 recognizes only isoforms of frataxin containing exon 4. On Western blots of normal human muscle, heart, cerebellum, and spinal cord extracts, MAB1594 recognizes a band migrating at approximately 18 kDa corresponding to processed frataxin (Campuzano, 1997). Slight cross reactivity with myosin may be observed by Western blot. Immunofluorescent labeling of HeLa cells with MAB1594 demonstrates that frataxin is predominantly localized in the mitochondria (Campuzano, 1997).
Isotype	IgG1κ
Species Reactivity	Human Mouse Rat
Species Reactivity Note	Mouse and rat. Expected to cross-react with human.
Antibody Type	Monoclonal Antibody
Entrez Gene Number	NM_000144.3
Entrez Gene Summary	This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified.
Gene Symbol	FXN FRDA FARR FA Fxn MGC57199 CyaY X25
Purification Method	Unpurified
UniProt Number	Q16595
UniProt Summary	FUNCTION: SwissProt: Q16595 # Probably involved in iron homeostasis. Anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production. SIZE: 210 amino acids; 23135 Da SUBUNIT: Monomer. SUBCELLULAR LOCATION: Mitochondrion. Note=Mitochondrial and extramitochondrial. TISSUE SPECIFICITY: Frataxin(81-210) is expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. PTM: Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein. Two forms exist, frataxin(56-210) and frataxin(81-210) which is the main form of mature frataxin. DISEASE: SwissProt: Q16595 # Defects in FXN are the cause of Friedreich ataxia (FA) [MIM:229300]. FA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. SIMILARITY: SwissProt: Q16595 ## Belongs to the frataxin family
Molecular Weight	~ 18 kDa

Biological Information

Immunogen

Full length human Frataxin fused to TrpE.

Epitope

exon 4

Clone

1G2

Concentration

Please refer to the Certificate of Analysis for the lot-specific concentration.

Host

Mouse

Specificity

Human Frataxin. MAB1594 recognizes only isoforms of frataxin containing exon 4. On Western blots of normal human muscle, heart, cerebellum, and spinal cord extracts, MAB1594 recognizes a band migrating at approximately 18 kDa corresponding to processed frataxin (Campuzano, 1997). Slight cross reactivity with myosin may be observed by Western blot. Immunofluorescent labeling of HeLa cells with MAB1594 demonstrates that frataxin is predominantly localized in the mitochondria (Campuzano, 1997).

Isotype

IgG1κ

Species Reactivity

Human
Mouse
Rat

Species Reactivity Note

Mouse and rat. Expected to cross-react with human.

Antibody Type

Monoclonal Antibody

Entrez Gene Number

NM_000144.3

Entrez Gene Summary

This nuclear gene encodes a mitochondrial protein which belongs to FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia. Alternative splicing occurs at this locus and two transcript variants encoding distinct isoforms have been identified.

Gene Symbol

FXN
FRDA
FARR
FA
Fxn
MGC57199
CyaY
X25

Purification Method

Unpurified

UniProt Number

Q16595

UniProt Summary

FUNCTION: SwissProt: Q16595 # Probably involved in iron homeostasis. Anti-apoptotic protein which prevents mitochondrial damage and reactive oxygen species (ROS) production.
SIZE: 210 amino acids; 23135 Da
SUBUNIT: Monomer.
SUBCELLULAR LOCATION: Mitochondrion. Note=Mitochondrial and extramitochondrial.
TISSUE SPECIFICITY: Frataxin(81-210) is expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
PTM: Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to mature size protein. Two forms exist, frataxin(56-210) and frataxin(81-210) which is the main form of mature frataxin.
DISEASE: SwissProt: Q16595 # Defects in FXN are the cause of Friedreich ataxia (FA) [MIM:229300]. FA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region.
SIMILARITY: SwissProt: Q16595 ## Belongs to the frataxin family

Molecular Weight

~ 18 kDa

Product Usage Statements
Quality Assurance	Routinely evaluated by Western Blot on PC12 lysates. Western Blot Analysis: 1:1000 dilution of this lot detected Frataxin on 10 μg of PC12 lysates.
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

Quality Assurance

Routinely evaluated by Western Blot on PC12 lysates.

Western Blot Analysis:
1:1000 dilution of this lot detected Frataxin on 10 μg of PC12 lysates.

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	Stable for 1 years at -20°C in undiluted aliquots from date of receipt. Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance

Storage and Shipping Information

Storage Conditions

Stable for 1 years at -20°C in undiluted aliquots from date of receipt.
Handling Recommendations: Upon first thaw, and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance

Packaging Information
Material Size	100 µL

Packaging Information

Material Size

100 µL

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

办公用品

Anti-CD45 (human), FITC, clone HI30

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