3S5E: Frataxin, mitochondrial

Crystal structure of human frataxin variant W155R, one of the Friedreich's ataxia point mutations. Determined by X-ray diffraction at 1.31 Å resolution. Released 29 Jun 2011.

Method: X-ray diffraction
Resolution: 1.31 Å
Organism: Homo sapiens
Chains: 1
Atoms: 1,126
Mol. weight: 14.19 kDa
Ligands: MG
Released: 29 Jun 2011

Explore 3S5E in 3D Show helices and sheets RCSB PDB PDBe

Secondary structure: helices and β-sheets

3S5E contains 2 α-helices and 8 β-strands across 1 chain. 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: 2 helices, 8 β-strands

Element	Residues	Length	Sheet
α-helix	92-113	22
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	162-166	5	1
β-strand	167-168	2	2
β-strand	173-174	2	2
β-strand	181	1	2
α-helix	182-194	13

Molecules and chains

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

Sequence of entity 1 (A), FASTA

>3S5E_1 Frataxin, mitochondrial (chains A)
GTLGHPGSLDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLG
TYVINKQTPNKQIRLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLS
SLAYSGKDA

Ligands and cofactors

ID	Name	Formula	Copies
MG	Magnesium ion	Mg	1

Primary citation

Friedreich's Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron-Sulfur Cluster Assembly Complex. Tsai, C.L., Bridwell-Rabb, J., Barondeau, D.P. Biochemistry (2011) 50:6478-6487. DOI 10.1021/bi200666h · 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
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…
3T3X 1.57 Å, 1.57 A structure of Friedreich's ataxia frataxin variant R165C
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…

Browse structure collections

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