Abstract:
:The relative frequencies of different subtypes of limb-girdle muscular dystrophies vary widely among different populations. We estimated the percentage of limb-girdle muscular dystrophy subtypes in Chinese people based on 68 patients with limb-girdle muscular dystrophy from the Myology Clinic, Neurology Department, First Hospital of Jilin University, China. A diagnosis of calpainopathy was made in 12 cases (17%), and dysferlin deficiency in 10 cases (15%). Two biopsies revealed α-sarcoglycan deficiency (3%), and two others revealed a lack of caveolin-3 (3%). A diagnosis of unclassified limb-girdle muscular dystrophy was made in the remaining patients (62%). The appearances of calpain 3- and dysferlin-deficient biopsies were similar, though rimmed vacuoles were unique to dysferlinopathy, while inflammatory infiltrates were present in both these limb-girdle muscular dystrophy type 2D biopsies. Macrophages were detected in seven dysferlinopathy biopsies. The results of this study suggest that the distribution of limb-girdle muscular dystrophy subtypes in the Han Chinese population is similar to that reported in the West. The less necrotic, regenerating and inflammatory appearance of limb-girdle muscular dystrophy type 2A, but with more lobulated fibers, supports the idea that calpainopathy is a less active, but more chronic disease than dysferlinopathy. Unusual features indicated an extended limb-girdle muscular dystrophy disease spectrum. The use of acid phosphatase stain should be considered in suspected dysferlinopathies. To the best of our knowledge, this is the first report to define the relative proportions of the various forms of limb-girdle muscular dystrophy in China, based on protein testing.
journal_name
Neural Regen Resjournal_title
Neural regeneration researchauthors
Mahmood OA,Jiang X,Zhang Qdoi
10.3969/j.issn.1673-5374.2013.20.010subject
Has Abstractpub_date
2013-07-15 00:00:00pages
1907-18issue
20eissn
1673-5374issn
1876-7958pii
NRR-8-1907journal_volume
8pub_type
杂志文章abstract::Although numerous studies have examined the neurotoxicity of acrylamide in adult animals, the effects on neuronal development in the embryonic and lactational periods are largely unknown. Thus, we examined the toxicity of acrylamide on neuronal development in the hippocampus of fetal rats during pregnancy. Sprague-Daw...
journal_title:Neural regeneration research
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journal_title:Neural regeneration research
pub_type: 杂志文章
doi:10.3969/j.issn.1673-5374.2013.23.001
更新日期:2013-08-15 00:00:00
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journal_title:Neural regeneration research
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更新日期:2021-03-01 00:00:00
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pub_type: 杂志文章
doi:10.4103/1673-5374.276359
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journal_title:Neural regeneration research
pub_type: 杂志文章
doi:10.3969/j.issn.1673-5374.2013.36.003
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journal_title:Neural regeneration research
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journal_title:Neural regeneration research
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journal_title:Neural regeneration research
pub_type: 已发布勘误
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