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Home / Assay Kits / ELISA Kits

ELISA Kit for Human Frataxin, mitochondrial

SKU : BHE13804551

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EIAAB SCIENCE INC, WUHAN

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Mfr.No.	E15247h
Alternative Names	Friedreich ataxia protein ; Fxn ; FRDA ; X25
Categories	Elisa
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.
Specificity	Natural and recombinant Human Frataxin, mitochondrial
Subcellular Location	Cytoplasm Mitochondrion PubMed:18725397 reports localization exclusively in mitochondria.
Entryname	FRDA_HUMAN
Platform	PF01491
Protein Name	Frataxin, mitochondrial
Sequence	50: MWTLGRRAVA ; GLLASPSPAQ ; AQTLTRVPRP ; AELAPLCGRR ; GLRTDIDATC ; 100: TPRRASSNQR ; GLNQIWNVKK ; QSVYLMNLRK ; SGTLGHPGSL ; DETTYERLAE ; 150: ETLDSLAEFF ; EDLADKPYTF ; EDYDVSFGSG ; VLTVKLGGDL ; GTYVINKQTP ; 200: NKQIWLSSPS ; SGPKRYDWTG ; KNWVYSHDGV ; SLHELLAAEL ; TKALKTKLDL ; 210: SSLAYSGKDA ;
Sequence Length	210
String	Q16595
Sub Unit	Monomer (probable predominant form). Oligomer. Monomers and polymeric aggregates of >1 MDa have been isolated from mitochondria. A small fraction of heterologous overexpressed recombinant frataxin forms high-molecular wight aggregates that incoroprate iron. Interacts with LYRM4 AND HSPA9. Interacts with ACO1. Interacts with ISCU isoform 1 and isoform 2. Interacts with FECH; one iron-bound FXN monomer seems to interact with a FECH homodimer. Interacts with SDHA and SDHB. Interacts with ACO2; the interaction is dependent on citrate.

Uniprot	Q16595
Gene Name	FXN
Immunogen Species	Homo Sapiens (Human)
Kegg	hsa:2395
Unigene	Hs.20685

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SKU	Supplier No.	Size	Your price	Quantity
BHE13804551	E15247h	96T	$736		Add to Cart

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