Frataxin antibody

Catalog No. ABIN1386148

Product Details anti-Frataxin Antibody

Target: Frataxin (FXN)

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Frataxin antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Cross-Reactivity: Human, Mouse, Rat

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Frataxin

Isotype: IgG

Application Details

Application Notes: WB 1:300-5000
IHC-P 1:200-400
IF(IHC-P) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Expiry Date: 12 months

Target Details for Frataxin

Target: Frataxin (FXN)

Alternative Name: Frataxin (FXN Products)

Synonyms: zgc:158218 antibody, LOC100284365 antibody, BcDNA:AT09528 antibody, CG8971 antibody, DFH antibody, Dfh antibody, Dmel\\CG8971 antibody, X25 antibody, dfh antibody, CyaY antibody, FA antibody, FARR antibody, FRDA antibody, RGD1565754 antibody, Frda antibody, frataxin antibody, fxn antibody, LOC100284365 antibody, TVAG_182150 antibody, fh antibody, FTX1 antibody, FXN antibody, Fxn antibody

Background:

Synonyms: X25, CyaY, d-FXN, FA antibody FARR, Frataxin mature form, Frataxin81-210, FRDA, FRDA_HUMAN, Friedreich ataxia protein, FXN, i-FXN, m56-FXN, m78-FXN, m81-FXN, MGC57199, MSF01, MSF31, MSF42.

Background: 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.

Pathways: Transition Metal Ion Homeostasis, Regulation of Cell Size