TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis

被引：21

作者：

Soraru, Gianni ^{[1
]}

Orsetti, Valeria ^{[1
]}

Buratti, Emanuele ^{[3
]}

Baralle, Francisco ^{[3
]}

Cima, Valentina ^{[1
]}

Volpe, Marco ^{[1
]}

D'Ascenzo, Carla ^{[1
]}

Palmieri, Arianna ^{[1
]}

Koutsikos, Kostantinos ^{[2
]}

Pegoraro, Elena ^{[1
]}

Angelini, Corrado ^{[1
]}

机构：

[1] Univ Padua, Dept Neurol Sci, Padua, Italy

[2] IRCSS San Camillo, Venice, Italy

[3] ICGEB, Trieste, Italy

来源：

AMYOTROPHIC LATERAL SCLEROSIS | 2010年 / 11卷 / 1-2期

关键词：

TDP-43; amyotrophic lateral sclerosis; skeletal muscle; FRONTOTEMPORAL LOBAR DEGENERATION; TAR-DNA-BINDING; MUTATIONS; PROGRANULIN; PROTEIN-43; DIAGNOSIS; NUCLEAR; DISEASE;

D O I：

10.3109/17482960902810890

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

TAR DNA binding protein (TDP-43) is the pathologic substrate of neuronal and glial aggregates in amyotrophic lateral sclerosis (ALS). Pathologic TDP-43 is hyperphosphorylated and cleaved to generate abnormal protein species that accumulate in the cytoplasm. To assess the hypothesis of TDP-43 pathology as a systemic disorder in ALS we analysed the immunohistochemical and biochemical profile of TDP-43 in muscle biopsies of 30 ALS patients and 30 controls. In all ALS muscle biopsies we observed that TDP-43 was constantly present in an intranuclear localization and TDP-43 Western blotting showed only a 43-KDa band as controls. Our results suggest that TDP-43 pathology is probably confined to the central nervous system in ALS.</.

引用

页码：240 / U35

页数：5

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