1LY7: Frataxin

The solution structure of the the c-terminal domain of frataxin, the protein responsible for friedreich ataxia. Determined by solution NMR. Released 26 Jun 2002.

Method: Solution NMR
Organism: Homo sapiens
Chains: 1
Atoms: 954
Mol. weight: 13.51 kDa
Released: 26 Jun 2002

Explore 1LY7 in 3D Show helices and sheets RCSB PDB PDBe

Secondary structure: helices and β-sheets

1LY7 contains 2 α-helices and 10 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, 10 β-strands

Element	Residues	Length	Sheet
α-helix	95-113	19
β-strand	122-126	5	1
β-strand	133-137	5	1
β-strand	141-146	6	1
β-strand	154-157	4	1
β-strand	158	1	2
β-strand	162	1	2
β-strand	165-166	2	1
β-strand	167-169	3	3
β-strand	172-174	3	3
β-strand	181	1	3
α-helix	182-193	12

Molecules and chains

Molecule	Chains	Type	Length	Organism	UniProt
frataxin	A	protein	121	Homo sapiens	Q16595 (AlphaFold model)

Sequence of entity 1 (A), FASTA

>1LY7_1 frataxin (chains A)
MDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQT
PNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLSSLAYSGKD
A

Primary citation

Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin. Musco, G., Stier, G., Kolmerer, B. et al. Structure (2000) 8:695-707. DOI 10.1016/S0969-2126(00)00158-1 · 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…
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

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