3T3X: Frataxin, mitochondrial

1.57 A structure of Friedreich's ataxia frataxin variant R165C. Determined by X-ray diffraction at 1.57 Å resolution. Released 10 Aug 2011.

Method: X-ray diffraction
Resolution: 1.57 Å
Organism: Homo sapiens
Chains: 2
Atoms: 2,082
Mol. weight: 28.48 kDa
Released: 10 Aug 2011

Explore 3T3X in 3D Show helices and sheets RCSB PDB PDBe

Secondary structure: helices and β-sheets

3T3X contains 7 α-helices and 15 β-strands across 2 chains. Residue ranges use author residue numbering, as in the PDB file, from PDBe. To see them in 3D, choose the Cartoon representation with Secondary structure coloring: helices, sheets and coils get different colors.

Chain A: 3 helices, 7 β-strands

Element	Residues	Length	Sheet
α-helix	92-112	21
α-helix	113-115	3
β-strand	124-128	5	1
β-strand	131-135	5	1
β-strand	142-148	7	1
β-strand	153-158	6	1
β-strand	162-168	7	1
β-strand	173-175	3	1
β-strand	181	1	1
α-helix	182-194	13

Chain B: 4 helices, 8 β-strands

Element	Residues	Length	Sheet
α-helix	92-111	20
α-helix	112-115	4
β-strand	124-128	5	2
β-strand	131-135	5	2
β-strand	142-148	7	2
α-helix	149-151	3
β-strand	153-158	6	2
β-strand	162-166	5	2
β-strand	167-168	2	3
β-strand	173-175	3	3
β-strand	180-181	2	3
α-helix	182-194	13

Molecules and chains

Molecule	Chains	Type	Length	Organism	UniProt
Frataxin, mitochondrial	A, B	protein	129	Homo sapiens	Q16595 (AlphaFold model)

Sequence of entity 1 (A, B), FASTA

>3T3X_1 Frataxin, mitochondrial (chains A, B)
GTLGHPGSLDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLG
TYVINKQTPNKQIWLSSPSSGPKCYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLS
SLAYSGKDA

Primary citation

Structure-Function Analysis of Friedreich's Ataxia Mutants Reveals Determinants of Frataxin Binding and Activation of the Fe-S Assembly Complex. Bridwell-Rabb, J., Winn, A.M., Barondeau, D.P. Biochemistry (2011) 50:7265-7274. DOI 10.1021/bi200895k · PubMed

Other PDB entries of the same protein (UniProt Q16595 (AlphaFold model), which also has an AlphaFold model), best resolution first:

3T3L 1.15 Å, 1.15 A structure of human frataxin variant Q153A
3T3K 1.24 Å, 1.24 A Structure of Friedreich's ataxia frataxin variant Q148R
3S4M 1.3 Å, Crystal structure of wild-type human frataxin
3S5E 1.31 Å, Crystal structure of human frataxin variant W155R, one of the Friedreich's ataxia point…
3T3T 1.38 Å, 1.38 A structure of human frataxin variant Q148G
3S5D 1.5 Å, Crystal structure of human frataxin variant W155A
3S5F 1.5 Å, Crystal structure of human frataxin variant W155F
9HO5 1.5 Å, Crystal Structure of the Human Frataxin protein in complex with a tailored Camelid…
3T3J 1.7 Å, 1.70 A structure of Friedreich's ataxia frataxin variant N146K
9HO4 1.76 Å, Crystal Structure of the Human Frataxin protein in complex with a tailored Camelid…
1EKG 1.8 Å, Mature human frataxin
9HO6 2.0 Å, Crystal Structure of the Human Frataxin protein in complex with a tailored Camelid…

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