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Frataxin Recombinant Rabbit Monoclonal Antibody [PSH15-18] - BSA and Azide free

Usd: 649

Specification

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Catalog# HA751547

Frataxin Recombinant Rabbit Monoclonal Antibody [PSH15-18] - BSA and Azide free

Application

  • WB

  • IHC-P

Reactivity

  • Human

  • Rat

With BSA and Azide
Conjugation

  • unconjugated

Overview

Product Name

Frataxin Recombinant Rabbit Monoclonal Antibody [PSH15-18] - BSA and Azide free

Antibody Type

Recombinant Rabbit monoclonal Antibody

Immunogen

Recombinant protein within human Frataxin aa 42-210.

Species Reactivity

Human, Rat

Validated Applications

WB, IHC-P

Molecular Weight

Predicted band size: 23 kDa

Positive Control

SW480 cell lysate, HCT 116 cell lysate, TF-1 cell lysate, Huh7 cell lysate, PC-12 cell lysate, human kidney tissue, human liver tissue.

Conjugation

unconjugated

Clone Number

PSH15-18

Product Features

Form

Liquid

Concentration

1mg/ml

Storage Instructions

Store at +4℃ after thawing. Aliquot store at -20℃. Avoid repeated freeze / thaw cycles.

Storage Buffer

1*PBS (pH7.4).

Isotype

IgG

Purification Method

Protein A affinity purified.

Application Dilution

  • WB

  • 1:2,000-1:5,000

  • IHC-P

  • 1:1,000

Target

Function

Frataxin is a protein that in humans is encoded by the FXN gene. It is located in the mitochondrion and Frataxin mRNA is mostly expressed in tissues with a high metabolic rate. The function of frataxin is not entirely clear, but it seems to be involved in assembly of iron-sulfur clusters. It has been proposed to act as either an iron chaperone or an iron storage protein. Frataxin mRNA is predominantly expressed in tissues with a high metabolic rate (including liver, kidney, brown fat and heart). Mouse and yeast frataxin homologues contain a potential N-terminal mitochondrial targeting sequence, and human frataxin has been observed to co-localise with a mitochondrial protein. Furthermore, disruption of the yeast gene has been shown to result in mitochondrial dysfunction. Friedreich's ataxia is thus believed to be a mitochondrial disease caused by a mutation in the nuclear genome (specifically, expansion of an intronic GAA triplet repeat in the FXN gene, which encodes the protein frataxin.).

Background References

1. Gao J et al. SIRT3 Regulates Clearance of Apoptotic Cardiomyocytes by Deacetylating Frataxin. Circ Res. 2023 Sep

2. Pignataro MF et al. Frataxin: from the sequence to the biological role. Biophys Rev. 2025 Apr

Subcellular Location

Mitochondrion; Cytoplasm, cytosol.

UNIPROT

SwissProt: Q16595 Human

SwissProt: O35943 Mouse

SwissProt: D3ZYW7 Rat

Synonyms

CyaY antibody

d-FXN antibody

FA antibody

FARR antibody

Frataxin mature form antibody

Frataxin(81-210) antibody

FRDA antibody

FRDA_HUMAN antibody

Friedreich ataxia protein antibody

Fxn antibody

Expand

Images

  • Western blot analysis of Frataxin on different lysates with Rabbit anti-Frataxin antibody (<a href="/products/HA751547" style="font-weight: bold;text-decoration: underline;">HA751547</a>) at 1/5,000 dilution.<br /><br />Lane 1: SW480 cell lysate (20 µg/Lane)<br />Lane 2: HCT 116 cell lysate (20 µg/Lane)<br />Lane 3: TF-1 cell lysate (20 µg/Lane)<br />Lane 4: Huh7 cell lysate (20 µg/Lane)<br />Lane 5: PC-12 cell lysate (20 µg/Lane)<br /><br />Predicted band size: 23 kDa<br />Observed band size: 14/19 kDa<br /><br />Exposure time: 3 minutes; ECL: K1801;<br />4-20% SDS-PAGE gel.<br /><br />Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (<a href="/products/HA751547" style="font-weight: bold;text-decoration: underline;">HA751547</a>) at 1/5,000 dilution was used in primary antibody dilution (<a href="/products/K1803" style="font-weight: bold;text-decoration: underline;">K1803</a>) at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (<a href="/products/HA1001" style="font-weight: bold;text-decoration: underline;">HA1001</a>) at 1/50,000 dilution was used for 1 hour at room temperature.

    Western blot analysis of Frataxin on different lysates with Rabbit anti-Frataxin antibody (HA751547) at 1/5,000 dilution.

    Lane 1: SW480 cell lysate (20 µg/Lane)
    Lane 2: HCT 116 cell lysate (20 µg/Lane)
    Lane 3: TF-1 cell lysate (20 µg/Lane)
    Lane 4: Huh7 cell lysate (20 µg/Lane)
    Lane 5: PC-12 cell lysate (20 µg/Lane)

    Predicted band size: 23 kDa
    Observed band size: 14/19 kDa

    Exposure time: 3 minutes; ECL: K1801;
    4-20% SDS-PAGE gel.

    Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (HA751547) at 1/5,000 dilution was used in primary antibody dilution (K1803) at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature.

  • Immunohistochemical analysis of paraffin-embedded human kidney tissue with Rabbit anti-Frataxin antibody (<a href="/products/HA751547" style="font-weight: bold;text-decoration: underline;">HA751547</a>) at 1/1,000 dilution.<br /><br />The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/HA751547" style="font-weight: bold;text-decoration: underline;">HA751547</a>) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

    Immunohistochemical analysis of paraffin-embedded human kidney tissue with Rabbit anti-Frataxin antibody (HA751547) at 1/1,000 dilution.

    The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (HA751547) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

  • Immunohistochemical analysis of paraffin-embedded human liver tissue with Rabbit anti-Frataxin antibody (<a href="/products/HA751547" style="font-weight: bold;text-decoration: underline;">HA751547</a>) at 1/1,000 dilution.<br /><br />The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/HA751547" style="font-weight: bold;text-decoration: underline;">HA751547</a>) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

    Immunohistochemical analysis of paraffin-embedded human liver tissue with Rabbit anti-Frataxin antibody (HA751547) at 1/1,000 dilution.

    The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (HA751547) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"