Recombinant Human Frataxin, mitochondrial (FxN)

Recombinant Human Frataxin, mitochondrial (FxN) — Modulates the RNA-binding activity of ACO1. Purity >85%.

SKU: BCRECP-000702 Category:

Product Specifications

Product SkuBCRECP-000702
Product DescriptionRecombinant Human Frataxin, mitochondrial (FxN) Protein is expressed from E.coli with N-terminal 10xHis-tagged and C-terminal Myc-tagged. It contains 1-210aa. [Accession | Q16595].
Uniprot No.Q16595
Gene NamesFxN
PurityGreater than 85% as determined by SDS-PAGE.
Expression SystemE.coli
Expression Region1-210aa
SpeciesHomo sapiens (Human)
Tag InfoN-terminal 10xHis-tagged and C-terminal Myc-tagged
Molecular weight28.1kDa
ActivityPlease contact us to obtain bioactivity data.
BufferIf the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
StorageStore at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Research AreasTags & Cell Markers

Function

Modulates the RNA-binding activity of ACO1. May be involved in the cytoplasmic iron-sulfur protein biogenesis. May contribute to oxidative stress resistance and overall cell survival.

Biological Context

Subcellular Location: Cytoplasm, cytosol
Tissue Specificity: Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts
Disease Association: Friedreich ataxia (FRDA) : 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, FRDA 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. [The disease is caused by variants affecting the gene represented in this entry]

Product Specifications

Recombinant Human Frataxin, mitochondrial (FxN) is a recombinant protein from Homo sapiens (Human), expressed in E.coli, covering amino acids 1-210aa, with N-terminal 10xHis-tagged and C-terminal Myc-tagged tag, molecular weight 28.1kDa, purity Greater than 85% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications.

Recommended applications depend on the target, product format, and product-specific validation data. Refer to the product datasheet or Certificate of Analysis (CoA), or contact BioCrest Sci for application guidance.

Biological Activity: Please contact us to obtain bioactivity data.

Storage: Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.

Shipping & Lead Time: Availability, shipping conditions, and estimated lead time are confirmed in the formal quotation or order confirmation based on the product format, destination, and order requirements.

Protein Biology

Function

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)

Subcellular Location

Cytoplasm, cytosol

Disease Association

Friedreich ataxia (FRDA) : 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, FRDA 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. [The disease is caused by variants affecting the gene represented in this entry]

Tissue Specificity

Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts

Subunit

Interacts with ACO1 (PubMed:20053667). Interacts with ISCU (cytoplasmic form) (PubMed:16091420)

Gene: FXN  |  Organism: Homo sapiens  |  Synonyms: Friedreich ataxia protein
Key Publications

Frequently Asked Questions

How do I order or inquire about this product?

Submit the Online Inquiry form with your required quantity and specifications, or email sales@biocrestsci.com. Available sizes, pricing, stock status, documentation, and estimated lead time are confirmed in the formal quotation.

What is the shipping and delivery time?

Availability, dispatch timing, shipping conditions, and delivery estimates depend on the product format, destination, and order requirements. These details are confirmed in the formal quotation or order confirmation.

How should this product be stored?

Follow the storage and handling instructions in the product-specific datasheet and Certificate of Analysis (CoA). Requirements may vary by product form and formulation; contact BioCrest Sci if product-specific guidance is needed.

What quality controls are performed on your products?

Quality-control data such as identity, purity, endotoxin level, or biological activity are provided only when specified on this product page, the datasheet, or Certificate of Analysis (CoA). Biological activity is not implied unless explicitly stated.

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