FXN Antibody

Datasheet
Code CSB-PA613687ZA01HU

Product Details

Full Product Name Rabbit anti-Homo sapiens (Human) FXN Polyclonal antibody
Uniprot No. Q16595
Target Names FXN
Alternative Names Frataxin, mitochondrial (EC 1.16.3.1) (Friedreich ataxia protein) (Fxn) [Cleaved into: Frataxin intermediate form (i-FXN); Frataxin(56-210) (m56-FXN); Frataxin(78-210) (d-FXN) (m78-FXN); Frataxin mature form (Frataxin(81-210)) (m81-FXN)], FXN, FRDA X25
Raised in Rabbit
Species Reactivity Homo sapiens
Immunogen Recombinant Human Frataxin, mitochondrial (FXN) (1-210AA)
Immunogen Species Homo sapiens (Human)
Conjugate Non-conjugated
Clonality Polyclonal
Isotype IgG
Purification Method Protein A/G
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form Liquid
Tested Applications ELISA, WB (ensure identification of antigen)
Protocols ELISA Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Made-to-order
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Target Data

Function Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. 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. 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. Modulates the RNA-binding activity of ACO1.
Involvement in disease Friedreich ataxia (FRDA)
Subcellular Location Mitochondrion, Cytoplasm, cytosol
Protein Families Frataxin family
Tissue Specificity Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Database Links

HGNC: 3951

OMIM: 229300

KEGG: hsa:2395

STRING: 9606.ENSP00000366482

UniGene: Hs.20685

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