**5.3.74 Testosterone**

20 Amyotrophic Lateral Sclerosis

It has been tested in several clinical trials without benefit for ALS patients (Jossan et al.,

Modified snake venom as neurotoxin did not show a benefit in ALS patients (Rivera et al.,

Sodium phenylbutyrate acts as a histone deacetylase inhibitor, therefore it improves

In a phase II study (NCT00107770), sodium phenylbutyrate was safe and well tolerated

Valproic acid (VPA) is a histone deacetylase inhibitor that showed antioxidative and antiapoptotic properties and reduced glutamate toxicity. It is used as antiepileptic drug. In a phase II clinical trial (NCT00136110), VPA has been found safe but not effective with

Talampanel is a derivated benzodiazepine which is a non-competitive antagonist at the

It has been evaluated in a phase II trial (NCT00982150) which showed safety and tolerability (Pascuzzi et al., 2010), whereas a subsequent phase II study (NCT00696332) did not show

The selective estrogen receptor modulator tamoxifen is an inhibitor of protein kinase C,

It has been evaluated in a phase II study (NCT00214110) in ALS patients (Brooks et al., 2005)

Currently, tamoxifen is evaluated in a phase II clinical trial (NCT01257581) in combination

In preclinical data, tauroursodeoxycholic acid (TUDCA) has been found to be neuroprotective, antioxidative, and antiapoptotic in rat models of neurodegenerative diseases such as stroke (Rodrigues et al., 2002) and Huntington's disease (Keene et al., 2002). TUDCA is currently evaluated in a phase II study (NCT00877604) for safety and efficacy.

TCH 346 showed neuroprotective effects by binding glyceraldehydes-3-phosphate

It has been evaluated in three phase II studies (NCT00230074, NCT00072709, NCT00036413),

and was recommended for a phase III trial (mentioned in Traynor et al., 2006).

The anti-depressant drug selegiline is an inhibitor of monoamino oxidase B.

**5.3.66 Selegiline** 

**5.3.67 Snake venom** 

**5.3.68 Sodium phenylbutyrate** 

(Cudkowicz et al., 2009).

**5.3.69 Sodium valproate** 

**5.3.70 Talampanel (GYKI 53405)** 

AMPA glutamate receptor.

with creatine in ALS patients.

**5.3.71 Tamoxifen** 

**5.3.73 TCH346** 

1980).

1994; Lange et al., 1998; Mazzini et al., 1994).

transcription and stimulates post-transcriptional pathways.

regard to survival and disease progression (Piepers et al., 2009).

efficacy in ALS patients (reviewed in Carlesi et al., 2011).

which is a mediator in neuroinflammation.

**5.3.72 Tauroursodeoxycholic acid (TUDCA)** 

dehydrogenase (GAPDH) (Mück-Seler & Pivac, 2000).

where it showed no beneficial effects in ALS (R. Miller et al., 2007).

The sexual hormone testosterone has been tested in a phase II study in combination with leuprolide (NCT00004771), the results are awaited.
