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1.
Acta Crystallogr C ; 68(Pt 7): i45-9, 2012 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-22763678

RESUMEN

The title phase (pentalead triindium pentabismuth heptadecasulfide) has been synthesized and structurally characterized. Its structure contains mixed Bi/In, In/Bi and Pb/In positions; all atoms lie on crystallographic mirror planes. The structural relationship between Pb(5)In(3)Bi(5)S(17) and a phase known by the formula Pb(4)In(3)Bi(7)S(18) is discussed. A comparatively large and complex structural `seed' is defined which is common to both phases. The structural changes within this seed when moving from one phase to the other are described by graphical means.

2.
Proc Natl Acad Sci U S A ; 91(11): 5089-93, 1994 May 24.
Artículo en Inglés | MEDLINE | ID: mdl-8197190

RESUMEN

Overaccumulation of abnormally organized mitochondria in so-called "ragged-red" skeletal muscle fibers is a morphological hallmark of mitochondrial myopathies, in particular of mitochondrial encephalomyopathies. Characteristic for the abnormal mitochondria is the occurrence of highly ordered crystalline inclusions. Immuno-electron microscopy revealed that these inclusions react heavily with specific antibodies against mitochondrial creatine kinase (Mi-CK). Image processing of selected crystalline inclusions, sectioned along the crystallographic b, c planes, resulted in an averaged picture displaying an arrangement of regular, square-shaped particles with a central cavity. The overall appearance, dimensions, and symmetry of these building blocks are very reminiscent of single isolated Mi-CK octamers. Taking these findings together, it is concluded that Mi-CK octamers indeed represent the major, if not the only, component of these mitochondrial inclusions.


Asunto(s)
Creatina Quinasa/metabolismo , Cuerpos de Inclusión/ultraestructura , Mitocondrias/enzimología , Miopatías Mitocondriales/enzimología , Humanos , Microscopía Inmunoelectrónica , Mitocondrias/ultraestructura , Miopatías Mitocondriales/patología
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