- Pathology of inherited manganese transporter deficiency.
Pathology of inherited manganese transporter deficiency.
Annals of neurology (2014-03-07)
Mirna Lechpammer, Michael S Clegg, Zukhrofi Muzar, Philip A Huebner, Lee-Way Jin, Sidney M Gospe
PMID24599576
ABSTRAKT
We followed a patient with manganese transporter deficiency due to homozygous SLC30A10 mutations from age 14 years until his death at age 38 years and present the first postmortem findings of this disorder. The basal ganglia showed neuronal loss, rhodanine-positive deposits, astrocytosis, myelin loss, and spongiosis. SLC30A10 protein was reduced in residual basal ganglia neurons. Depigmentation of the substantia nigra and other brainstem nuclei was present. Manganese content of basal ganglia and liver was increased 16-fold and 9-fold, respectively. Our study provides a pathological foundation for further investigation of central nervous system toxicity secondary to deregulation of manganese metabolism.
MATERIAŁY
Numer produktu
Marka
Opis produktu
Manganese, foil, not light tested, 50x50mm, thickness 0.01mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.025mm, permanent polyester support, 98.7%
Manganese, foil, 25x25mm, thickness 2mm, hot-pressed, 99.95%
Manganese, foil, not light tested, 25x25mm, thickness 0.004mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.001mm, permanent polyester support, 98.7%
Manganese, foil, 50x50mm, thickness 2mm, hot-pressed, 99.95%
Manganese, foil, not light tested, 50x50mm, thickness 0.015mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.005mm, permanent polyester support, 98.7%
Manganese, rod, 10mm, diameter 4.0mm, cast, 99.5%
Manganese, rod, 50mm, diameter 4.0mm, cast, 99.5%
Manganese, foil, not light tested, 50x50mm, thickness 0.0025mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.0025mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.0125mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.008mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.002mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.006mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.01mm, permanent polyester support, 98.7%
Manganese, rod, 50mm, diameter 6.35mm, cast, 99.5%
Manganese, foil, not light tested, 50x50mm, thickness 0.003mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.007mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.025mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.004mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.009mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.02mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.008mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.005mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.05mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.009mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 50x50mm, thickness 0.006mm, permanent polyester support, 98.7%
Manganese, foil, not light tested, 25x25mm, thickness 0.001mm, permanent polyester support, 98.7%