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Therapeutic strategies for spinal and bulbar muscular atrophy (SBMA)


, : Therapeutic strategies for spinal and bulbar muscular atrophy (SBMA). Drugs of the Future 32(10): 907-917

Spinal and bulbar muscular atrophy (SBMA) is an adult-onset neurodegenerative disease characterized by slowly progressive weakness, atrophy of bulbar, facial and limb muscles, and mild androgen insensitivity. The cause of this disease is expansion of a trinucleotide CAG repeat which encodes the polyglutamine tract within the first exon of the androgen receptor (AR) gene. SBMA occurs exclusively in adult males, whereas both heterozygous and homozygous females are usually asymptomatic. Lower motor neurons in the anterior horn of the spinal cord and those in the brainstem motor nuclei are predominantly affected in SBMA, and other neuronal and non-neuronal tissues are also widely involved to a lesser extent. SBMA is considered an intractable disease, but several therapeutic approaches have been developed based on new insight into the pathogenesis. There are several lines of evidence indicating that testosterone, the ligand for ARs, plays a crucial role in the pathogenesis of neurodegeneration in SBMA, leading to clinical trials of androgen deprivation therapies. Moreover, animal studies have revealed other key molecules in the pathogenesis of SBMA, such as heat shock proteins (HSPs), transcriptional co-activators and axon motors, suggesting additional therapeutic targets.

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