Anti-FXN monoclonal antibody, clone 29B6EC2 [Alexa Fluor® 488]

Antibody Isotype

IgG1

Clone

29B6EC2

Species Reactivity

Human

Immunogen

Recombinant full length mature protein, corresponding to amino acids 56-210 of Human Frataxin (UniProt: Q16595).

Conjugate

Application Notes

Flow Cyt: 0.2 μg/ml.

Alternative Names

FXN; frataxin; FRDA, Friedreich ataxia; frataxin, mitochondrial; CyaY; FA; FARR; X25; Friedreich ataxia protein; FRDA; MGC57199;

Entrez Gene ID

UniProt ID

Antigen Description

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.

Pathway

HIF-2-alpha transcription factor network, organism-specific biosystem; Metabolism of proteins, organism-specific biosystem; Mitochondrial Protein Import, organism-specific biosystem;