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AB176112

人Frataxin ELISA试剂盒

Human Frataxin ELISA Kit

5

(2 Reviews)

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(7 Publications)

Human Frataxin ELISA Kit is a single-wash 90-min Simplestep used to quantify Human Frataxin with a sensitivity of 2.1 pg/mL. The assay uses a simple mix-wash-read protocol with just one incubation and one wash step.

- Colorimetric Sandwich ELISA - 450 nm readout : works on any standard plate reader
- Different formats for different needs: 384-well plate for higher throughput
- Design your own immunoassay: we also offer the conjugation-ready antibody pair

查看别名

FRDA, X25, FXN, Friedreich ataxia protein, Fxn

11 Images
ELISA - Human Frataxin ELISA Kit (AB176112)
  • ELISA

Supplier Data

ELISA - Human Frataxin ELISA Kit (AB176112)

Quantification of Frataxin expression in different cell lines.

Interpolated values of Frataxin are plotted for the indicated cell lines based on a extract load of 10 μg/mL.

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Representative image of Human Frataxin ELISA Kit (ab176112)

Components shown from left to right :

- 10X Wash Buffer PT (ab206977)

- 10X Human Frataxin ELISA Kit Capture Antibody

- 10X Human Frataxin Detector Antibody

- 10X Human Frataxin ELISA Kit Lyophilised Purified Protein (2 vials)

- Human Frataxin ELISA Kit 50X Cell Extraction Enhancer Solution (ab193971)

- Sample diluent NS

- TMB solution

- Stop Solution

- 5X Cell Extraction Buffer PTR (ab193970)

- Antibody Diluent 4BI

SimpleStep Pre-Coated 96-Well Microplate (ab206978) - shown behind bottles / vials.

Plate Seals - shown underneath bottles / vials.

Note : The vial labels shown in this image use generic names for illustrative purposes only and may not exactly match the specific component names included in the ELISA kit.

Note : Colors of solutions in image may not precisely match the shade of colors in the actual kit

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Lab

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Interpolated concentration of native Frataxin was measured in duplicate at different sample concentrations in 96-well and 384-well plates. Undiluted samples are 50 µg/mL HeLa cell lysate. The interpolated dilution factor corrected values are plotted (mean +/- SD, n=2). Sample dilutions are made in 1X Cell Extraction Buffer PTR.

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Example of Frataxin standard curve.

Background-subtracted data values (mean +/- SD) are graphed.

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Raw data value for example of frataxin standard curve.

Raw data values are shown in the table

ELISA - Human Frataxin ELISA Kit (AB176112)
  • ELISA

Supplier Data

ELISA - Human Frataxin ELISA Kit (AB176112)

Titration of HeLa cell extract within the working range of the assay.

Background subtracted data from duplicate measurements are plotted.

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Lab

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Example of Human Frataxin standard curve in 96-well and 384-well plate. Background-subtracted data values (mean +/- SD) are graphed.

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Unknown

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Linearity of dilution for Recombinant Frataxin.

Recombinant Frataxin was measured in biological samples in a 2-fold dilution series

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Supplier Data

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Quantification of Frataxin expression in different human patient samples.

Transformed B lymphocyte cells from Friedreich's Ataxia (FRDA) samples, due to a stable homozygous GAA repeat insertion, are compared to heterozygous carrier B lymphocyte cells and control B lymphocyte cells. B lymphocyte cell extracts were analyzed across a 7-point titration (0.1-100 μg/mL) and frataxin levels were interpolated from the standard curve. Average interpolated values of Frataxin are plotted.

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

AbReview

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

Standard curve generated using Abcam's Frataxin Human SimpleStep ELISA™ Kit (ab176112) with human brain tissue homogenate mixed in different proportions with mouse brain tissue homogenate. ab176112 did not cross-react with mouse frataxin. Both kinetic and endpoint readouts were used sequentially for a larger dynamic range. ab110353, 12ng human frataxin full length protein was also used to extended the standard curve to 12ng/ml.

This image is courtesy of an anonymous Abreview

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)
  • sELISA

Lab

Sandwich ELISA - Human Frataxin ELISA Kit (AB176112)

SimpleStep ELISA technology allows the formation of the antibody-antigen complex in one single step, reducing assay time to 90 minutes. Add samples or standards and antibody mix to wells all at once, incubate, wash, and add your final substrate. See protocol for a detailed step-by-step guide.

关键信息

检测方法

Colorimetric

样品类型

Cell culture extracts, Tissue Extracts

反应种属

Human

检测类型

Sandwich (quantitative)

灵敏度

= 2.1 pg/mL

范围

9.88 - 800 pg/mL

检测时间

1h 30m

检测平台

Microplate

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反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "sELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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AB256593

Human Frataxin Antibody Pair - BSA and Azide free

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我们建议您务必查阅产品说明书，以确认其是否符合您的实验需求；若有疑问，也可联系我们的技术团队获取协助。

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产品详情

Human Frataxin ELISA Kit ab176112 is a rapid single-wash 90-min ELISA kit to measure Human Frataxin in cell culture extracts and tissue extracts. This SimpleStep ELISA® sensitivity is 2.1 pg/mL.

How the assay works

Human Frataxin SimpleStep ELISA® employs capture antibodies conjugated to an affinity tag that is recognized by the monoclonal antibody used to coat our SimpleStep ELISA® plates. This approach to sandwich ELISA allows the formation of the antibody-analyte sandwich complex in a single step, significantly reducing assay time. See the SimpleStep ELISA® protocol summary in the image section for further details.

Assay Specificity
This kit recognizes both native and recombinant human Frataxin protein in cell and tissue extract samples only. Serum, plasma, saliva, and urine samples have not been tested with this kit.

Human Frataxin ELISA Kit ab176112 protocol summary:
1. Mix: add samples/standards to the wells together with the capture and detector antibody cocktail. Incubate for 1 hr at room temperature
2. Wash
3. Add TMB development solution - incubate for 10 min
4. Add stop solution
5. Read the results on a plate reader at 450nm

How other researchers are using Human Frataxin ELISA Kit ab176112
Human Frataxin ELISA Kit ab176112 has been used to study effects and mechanism of Friedreich ataxia including cardiac mitochondrial stress(1), treatment with trans-activator of transcription peptides(2), and treatment with cytokine therapy (3).

References:
(1)Giovanni Manfredi et al. 2023, PMID: 37691621,
(2)Joaquim Ros et al. 2018, PMID: 28980774,
(3)Alastair Wilkins et al. 2017, PMID: 28009062

Save your precious samples
Our 384-well Human Frataxin ELISA Kit only requires a maxmum of 12.5µL

Design your own immunoassay
We offer the antibody pair used in this kit in a BSA and Azide-free format, ready for conjugation
- Human Frataxin Antibody Pair - BSA and Azide free ab256593
- Anti-Frataxin antibody [30-17A11AC7] - BSA and Azide free (Capture) ab259433
- Anti-Frataxin antibody [30-18A5DB1] - BSA and Azide free (Detector) ab259434

Related and recommended products
Human Frataxin ELISA Kit ab176112 is commonly used to study Friedreich's ataxia, a neurodegenerative disease caused by frataxin mutations leading to oxidative stress.

Other products to measure frataxin include:
- Mouse Frataxin ELISA Kit ab199078
- Anti-Frataxin antibody [17A11] ab113691
- Recombinant Human Frataxin protein ab95502

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精确度

[ { "reproducibilityType": "Inter", "sample": "HeLa", "replicates": 3, "mean": null, "standardDeviation": null, "coefficientOfVariability": "6.3" }, { "reproducibilityType": "Intra", "sample": "HeLa", "replicates": 9, "mean": null, "standardDeviation": null, "coefficientOfVariability": "2.3" } ]
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回收率

[ { "sample": "Cell culture media", "range": "93 - 101 %", "average": "= 97" }, { "sample": "EDTA Plasma", "range": "88 - 92 %", "average": "= 89" }, { "sample": "Serum", "range": "86 - 98 %", "average": "= 92" } ]
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规格

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性能和储存信息

运输条件
Blue Ice
推荐的短期储存条件
+4°C
推荐的长期储存条件
+4°C
储存信息
+4°C
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补充信息

This supplementary information is collated from multiple sources and compiled automatically.

Frataxin often known by the alternate name FXN is a mitochondrial protein with a mass of approximately 21000 Dalton. It is expressed mainly in tissues with high energy demands like the heart liver and pancreas. Frataxin plays an important role in iron-sulfur cluster assembly which is essential for various cellular processes. The protein is a part of mitochondria where it regulates iron homeostasis and prevents oxidative damage by minimizing iron-induced free radical generation.
Biological function summary

Several cellular processes depend on the correct function of this protein. Frataxin assists in forming iron-sulfur clusters acting within a multiprotein complex in the mitochondria. The complex includes proteins such as ISCU which are involved in the assembly and repair of iron-sulfur clusters. These clusters are necessary for supporting mitochondrial electron transport and other fundamental metabolic pathways that require iron-sulfur dependencies.

Pathways

Frataxin's involvement extensively affects the mitochondrial respiratory chain and the mitochondrial biogenesis process. It plays a role in the electron transport chain by stabilizing iron-sulfur-containing complexes. NAB is one associated protein that interacts closely within these pathways sharing a connection through iron-sulfur cluster transportation and assembly systems. Efficient function of these pathways ensures a proper energetic output of cells.

Frataxin mutations are directly linked to Friedreich's ataxia a neurodegenerative disease causing progressive damage to the nervous system. The deficiency or dysfunction in frataxin causes accumulation of iron in mitochondria leading to increased oxidative stress. Another related disorder includes heart disease which emerges due to the same oxidative stress pathway. Proteins such as Nfs1 are also involved sharing the responsibility with frataxin in scavenging excess iron protecting against related tissue damage.
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产品实验方案

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靶点信息

Frataxin mature form. Functions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the [2Fe-2S] cluster assembly (PubMed : 12785837, PubMed : 24971490). Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release (PubMed : 24971490). Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly (PubMed : 29576242). The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1 : LYRM4 : NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. Then this complex is stabilized by FDX2 which provides reducing equivalents to accomplish the [2Fe-2S] cluster assembly. Finally, the [2Fe-2S] cluster is transferred from ISCU to chaperone proteins, including HSCB, HSPA9 and GLRX5 (By similarity). May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity (PubMed : 15641778). May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems (PubMed : 11823441, PubMed : 12755598). May function as an iron chaperone protein that protects the aconitase [4Fe-4S]2+ cluster from disassembly and promotes enzyme reactivation (PubMed : 15247478). May play a role as a high affinity iron binding partner for FECH that is capable of both delivering iron to ferrochelatase and mediating the terminal step in mitochondrial heme biosynthesis (PubMed : 15123683, PubMed : 16239244).. Extramitochondrial frataxin. Modulates the RNA-binding activity of ACO1 (PubMed : 20053667). May be involved in the cytoplasmic iron-sulfur protein biogenesis (PubMed : 16091420). May contribute to oxidative stress resistance and overall cell survival (PubMed : 16608849).
See full target information FXN
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文献 (7)

Recent publications for all applications. Explore the full list and refine your search

Disease models & mechanisms 16: PubMed37691621

2023

Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency.

Applications

Unspecified application

Species

Unspecified reactive species

Nicole M Sayles,Jill S Napierala,Josef Anrather,Nadège Diedhiou,Jixue Li,Marek Napierala,Hélène Puccio,Giovanni Manfredi

Genes 14: PubMed37628705

2023

Finding an Appropriate Mouse Model to Study the Impact of a Treatment for Friedreich Ataxia on the Behavioral Phenotype.

Applications

Unspecified application

Species

Unspecified reactive species

Camille Bouchard,Catherine Gérard,Solange Gni-Fiene Yanyabé,Nathalie Majeau,Malek Aloui,Gabrielle Buisson,Pouiré Yameogo,Vanessa Couture,Jacques P Tremblay

Molecular therapy. Methods & clinical development 19:120-138 PubMed33209958

2020

High Levels of Frataxin Overexpression Lead to Mitochondrial and Cardiac Toxicity in Mouse Models.

Applications

Unspecified application

Species

Unspecified reactive species

Brahim Belbellaa,Laurence Reutenauer,Nadia Messaddeq,Laurent Monassier,Hélène Puccio

Molecular therapy : the journal of the American So 26:1940-1952 PubMed29853274

2018

Rapid and Complete Reversal of Sensory Ataxia by Gene Therapy in a Novel Model of Friedreich Ataxia.

Applications

Unspecified application

Species

Unspecified reactive species

Françoise Piguet,Charline de Montigny,Nadège Vaucamps,Laurence Reutenauer,Aurélie Eisenmann,Hélène Puccio

Bioorganic & medicinal chemistry 26:3359-3369 PubMed29773347

2018

Lipophilic methylene blue analogues enhance mitochondrial function and increase frataxin levels in a cellular model of Friedreich's ataxia.

Applications

Unspecified application

Species

Unspecified reactive species

Omar M Khdour,Indrajit Bandyopadhyay,Sandipan Roy Chowdhury,Nishant P Visavadiya,Sidney M Hecht

Journal of cellular and molecular medicine 22:834-848 PubMed28980774

2017

Frataxin-deficient neurons and mice models of Friedreich ataxia are improved by TAT-MTScs-FXN treatment.

Applications

Unspecified application

Species

Unspecified reactive species

Elena Britti,Fabien Delaspre,Anat Feldman,Melissa Osborne,Hagar Greif,Jordi Tamarit,Joaquim Ros

Annals of neurology 81:212-226 PubMed28009062

2016

Cytokine therapy-mediated neuroprotection in a Friedreich's ataxia mouse model.

Applications

sELISA

Species

Human

Kevin C Kemp,Nadia Cerminara,Kelly Hares,Juliana Redondo,Amelia J Cook,Harry R Haynes,Bronwen R Burton,Mark Pook,Richard Apps,Neil J Scolding,Alastair Wilkins
View all publications
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