1LY7

The solution structure of the the c-terminal domain of frataxin, the protein responsible for friedreich ataxia

Replaces:  1DLX

Summary for 1LY7

• Entry DOI: 10.2210/pdb1ly7/pdb
• Related: 1dlx
• NMR Information: BMRB: 4342
• Descriptor: frataxin (1 entity in total)
• Functional Keywords: alpha-beta, unknown function
• Biological source: Homo sapiens (human)
• Cellular location: Cytoplasm: Q16595
• Total number of polymer chains: 1
• Total formula weight: 13505.93

Authors

Musco, G.,Stier, G.,Kolmerer, B.,Adinolfi, S.,Martin, S.,Frenkiel, T.,Gibson, T.,Pastore, A. (deposition date: 2002-06-07, release date: 2002-06-26, Last modification date: 2024-05-22)

Primary citation

Musco, G.,Stier, G.,Kolmerer, B.,Adinolfi, S.,Martin, S.,Frenkiel, T.,Gibson, T.,Pastore, A.
Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin
Structure Fold.Des., 8:695-707, 2000

PubMed Abstract: Lesions in the gene for frataxin, a nuclear-encoded mitochondrial protein, cause the recessively inherited condition Friedreich's ataxia. It is thought that the condition arises from disregulation of mitochondrial iron homeostasis, with concomitant oxidative damage leading to neuronal death. Very little is, as yet, known about the biochemical function of frataxin.

PubMed: 10903947
DOI: 10.1016/S0969-2126(00)00158-1

Experimental method

SOLUTION NMR