Fxn - frataxin Gene

Also Known as FA; X25; FARR; Frda

Species: Mus musculus

Gene Type: protein coding

Gene ID: 14297

Summary

Predicted to enable several functions, including 2 iron, 2 sulfur cluster binding activity; ferroxidase activity; and iron ion binding activity. Acts upstream of or within several processes, including adult walking behavior; negative regulation of organ growth; and proprioception. Located in mitochondrion. Is expressed in several structures, including brain; cartilage condensation; heart; hemolymphoid system; and lower jaw incisor. Used to study Friedreich ataxia. Human ortholog(s) of this gene implicated in Friedreich ataxia; Friedreich ataxia 1; and type 2 diabetes mellitus. Orthologous to human FXN (frataxin). [provided by Alliance of Genome Resources, Apr 2022]

Fxn Products (1)

mRNA	Protein	Name
NM_008044.3	NP_032070.1	frataxin, mitochondrial precursor

Gene Ontology

Biological Process
Cellular Component

Biological Process GO Annotation	Evidence	References	Source
involved in [4Fe-4S] cluster assembly	IDA IDA: Inferred from direct assay	25597503	MGI
acts upstream of or within adult walking behavior	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of or within aerobic respiration	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of or within embryo development ending in birth or egg hatching	IMP IMP: Inferred from mutant phenotype	10767347	MGI
acts upstream of or within intracellular iron ion homeostasis	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of or within iron-sulfur cluster assembly	IMP IMP: Inferred from mutant phenotype	16278235	MGI
acts upstream of or within mitochondrion organization	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of or within muscle cell cellular homeostasis	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of or within negative regulation of multicellular organism growth	IMP IMP: Inferred from mutant phenotype	17404227	MGI
acts upstream of or within negative regulation of organ growth	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of organ growth	IMP IMP: Inferred from mutant phenotype	9405681	MGI
acts upstream of or within oxidative phosphorylation	IMP IMP: Inferred from mutant phenotype	16278235	MGI
acts upstream of or within proprioception	IMP IMP: Inferred from mutant phenotype	11175786	MGI

Cellular Component GO Annotation	Evidence	References	Source
part of mitochondrial [2Fe-2S] assembly complex	IDA IDA: Inferred from direct assay	21298097	MGI
located in mitochondrion	IDA IDA: Inferred from direct assay	17597094	MGI

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

Protein Preferred Names	Protein Names
frataxin, mitochondrial	Friedreich ataxia

Recombinant Fxn Proteins

Cat. No.	Product Name	Accession	Purity
HY-P71735	FXN Protein, Mouse (P.pastoris, His)	O35943 (L78-T207)	≥ 90%, as determined by reducing SDS-PAGE.
HY-P72199	FXN Protein, Mouse (His)	O35943 (L78-T207)	≥ 90%, as determined by reducing SDS-PAGE.

Related Agents

Approval
Clinical Phase
Pre-clinical Phase

Approval

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-B0364A	Dyclonine hydrochloride		99.12%	Unkown

Clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Phase
HY-149737	FXn		/	Phase 1

Pre-clinical Phase

Bioactive Molecules (5)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS05162	FXN Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-B0364AS	Dyclonine-d9 hydrochloride		/	Unkown
HY-158740	1(R)-(Trifluoromethyl)oleyl alcohol	Inhibitor	/	Unkown
HY-RS18448	Fxn Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS24930	Fxn Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Homo sapiens	Fxn	NCBI	NCBI:2395