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Therapeutic potential of Liuwei dihuang wang, a traditional Chinese Medicine, for spinal muscular atrophy in vitro and in vivo
Author(s) -
Lo YiChing,
Tseng YuTing,
Liang WeiFang,
Jong YuhJyh
Publication year - 2013
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.27.1_supplement.lb515
Subject(s) - sma* , spinal muscular atrophy , motor neuron , gene knockdown , blot , in vivo , motor function , muscle atrophy , viability assay , spinal cord , atrophy , medicine , biology , anatomy , pathology , cell , neuroscience , cell culture , biochemistry , physical medicine and rehabilitation , genetics , mathematics , microbiology and biotechnology , combinatorics , gene
Spinal muscular atrophy (SMA) is a neurodegenerative disease caused by abnormally low cellular levels of the survival motor neuron (SMN) protein, resulting in motor neuron degeneration, muscle atrophy and loss of motor function. Currently, there is no effective treatment for SMA. Liuwei dihung wan (LDW) is a traditional Chinese medicine used for neurosis, diabetics and renal disorders. The aim of this study is to evaluate the therapeutic potential of LDW for SMA using inducible SMN ‐knockdown NSC34 motor neuron cells and SMA‐like mice (SMNΔ7). Protein expression was detected by western blots. Cell viability was determined by MTT and LDH assay. SMA‐like mice were assessed daily for survival and body weight. Motor function was measured by hind‐limb suspension test. In SMN ‐knockdown NSC34 motor neurons, LDW increased SMN expression and cell viability. In SMA‐like mice, LDW improved survival, body weight and hind‐limb scores. Moreover, LDW increased SMN expression in brain, spinal cord and gastrocnemius muscle of SMA mice. In conclusion, LDW could enhance SMN expression, diminish loss of motor neurons, and improve motor function and survival of SMA mice, suggesting LDW is a potential candidate for SMA treatment. This study was supported by grant (no. NSC 99–2320‐B‐037–023‐MY3) provided from National Science Council of Taiwan.