Structure of frataxin iron cores: An X-ray absorption spectroscopic study

被引：57

作者：

Nichol, H

Gakh, O

O'Neill, HA

Pickering, IJ

Isaya, G

George, GN

机构：

[1] Univ Saskatchewan, Dept Cell Biol & Anat, Coll Med, Saskatoon, SK S7N 5E5, Canada

[2] Mayo Clin & Mayo Fdn, Dept Pediat & Adolescent Med, Rochester, MN 55905 USA

[3] Mayo Clin & Mayo Fdn, Dept Biochem & Mol Biol, Rochester, MN 55905 USA

[4] Stanford Synchrotron Radiat Lab, Stanford Linear Accelerator Ctr, Menlo Pk, CA 94025 USA

来源：

BIOCHEMISTRY | 2003年 / 42卷 / 20期

关键词：

D O I：

10.1021/bi027021l

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

X-ray absorption spectroscopy at the iron K-edge indicates that the iron cores of human and yeast frataxin polymers assembled in vitro are identical to each other and are similar but not identical to ferritin cores. Both frataxin polymers contain ferrihydrite, a biomineral composed of ferric oxide/hydroxide octahedra. The ferrihydrite in frataxin is less ordered than iron cores of horse spleen ferritin, having fewer face-sharing Fe-Fe interactions but similar double corner-sharing interactions. The extended X-ray absorption fine structure (EXAFS) analysis agrees with previous electron microscopy data showing that frataxin cores are composed of very small ferrihydrite crystallites.

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收藏

页码：5971 / 5976

页数：6

相关论文

共 53 条

[1] Iron-dependent self assembly of recombinant yeast frataxin: Implications for Friedreich ataxia [J].

Adamec, J ;

Rusnak, F ;

Owen, WG ;

Naylor, S ;

Benson, LM ;

Gacy, AM ;

Isaya, G .

AMERICAN JOURNAL OF HUMAN GENETICS, 2000, 67 (03) :549-562

[2] Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin [J].

Babcock, M ;

deSilva, D ;

Oaks, R ;

DavisKaplan, S ;

Jiralerspong, S ;

Montermini, L ;

Pandolfo, M ;

Kaplan, J .

SCIENCE, 1997, 276 (5319) :1709-1712

[3] Aggregation-based crystal growth and microstructure development in natural iron oxyhydroxide biomineralization products [J].

Banfield, JF ;

Welch, SA ;

Zhang, HZ ;

Ebert, TT ;

Penn, RL .

SCIENCE, 2000, 289 (5480) :751-754

[4] Erythroid differentiation and protoporphyrin IX down-regulate frataxin expression in Friend cells: characterization of frataxin expression compared to molecules involved in iron metabolism and hemoglobinization [J].

Becker, EM ;

Greer, JM ;

Ponka, P ;

Richardson, DR .

BLOOD, 2002, 99 (10) :3813-3822

[5] Regulation of plant ferritin synthesis:: how and why [J].

Briat, JF ;

Lobréaux, S ;

Grignon, N ;

Vansuyt, G .

CELLULAR AND MOLECULAR LIFE SCIENCES, 1999, 56 (1-2) :155-166

[6] Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion [J].

Campuzano, V ;

Montermini, L ;

Molto, MD ;

Pianese, L ;

Cossee, M ;

Cavalcanti, F ;

Monros, E ;

Rodius, F ;

Duclos, F ;

Monticelli, A ;

Zara, F ;

Canizares, J ;

Koutnikova, H ;

Bidichandani, SI ;

Gellera, C ;

Brice, A ;

Trouillas, P ;

DeMichele, G ;

Filla, A ;

DeFrutos, R ;

Palau, F ;

Patel, PI ;

DiDonato, S ;

Mandel, JL ;

Cocozza, S ;

Koenig, M ;

Pandolfo, M .

SCIENCE, 1996, 271 (5254) :1423-1427

[7] Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae [J].

Cavadini, P ;

Gellera, C ;

Patel, PI ;

Isaya, G .

HUMAN MOLECULAR GENETICS, 2000, 9 (17) :2523-2530

[8] Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia [J].

Cavadini, P ;

O'Neill, HA ;

Benada, O ;

Isaya, G .

HUMAN MOLECULAR GENETICS, 2002, 11 (03) :217-227

[9] Mineralization in ferritin: An efficient means of iron storage [J].

Chasteen, ND ;

Harrison, PM .

JOURNAL OF STRUCTURAL BIOLOGY, 1999, 126 (03) :182-194

[10] ABINITIO CURVED-WAVE X-RAY-ABSORPTION FINE-STRUCTURE [J].

DELEON, JM ;

REHR, JJ ;

ZABINSKY, SI ;

ALBERS, RC .

PHYSICAL REVIEW B, 1991, 44 (09) :4146-4156

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