FXN pAb

Cat no: BS6609

Supplier: Bioworld Technology, Inc.

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Friedreich ataxia is a progressive neurodegenerative disorder caused by loss of function mutations in the frataxin gene. The human frataxin gene maps to chromosome 9q13.The frataxin gene encodes a mitochondrial protein of the same name. Frataxin assembles into a stable homopolymer with iron-binding capabilities. When expressed in E. Coli, human frataxin binds iron atoms at a rate of 10 iron atoms per 1 molecule of the frataxin polymer. Thus, frataxin appears to function in some capacity for iron-storage for the mitochondria. Frataxin may also function as an activator of oxidative phosphorylation to increase mitochondrial membrane potential and elevate cellular ATP. Frataxin is expressed in tissues with high metabolic activity including heart, liver and brown fat.

Catalogue number:	BS6609
Reactivities:	Human, Mouse, Rat
Hosts:	Rabbit
Applications:	Immunohistochemistry, Western Blot
Size:	100ug/100ul
Swiss prot:	Q16595
Dilutions:	WB: 1:500~1:2000 IHC: 1:50~1:200
Form:	liquid
Storage buffer:	1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration:	1ug/ul
Antigen:	Recombinant full length Human FXN.
Species:	FXN pAb detects endogenous levels of FXN protein.
Molecular weight:	~ 23 kDa
Purity:	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp:	Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names:	CyaY; d-FXN; FA; FARR; Frataxin mature form; Frataxin(81-210); FRDA; FRDA; Friedreich ataxia protein; Fxn; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; X25;
Additional info:	For research use only, not for use in diagnostic procedure.

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FXN pAb

Cat no: BS6609

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