Spirit of the Coast Analytics

The SOTC Analytics Mission

SOTC Analytics strives to report medical intelligence pertaining to neurodegenerative & neurodevelopmental disorders to include Alzheimer's Disease, Dementia with Lewy Bodies, Parkinson's Disease, Rett Syndrome, Angelman Syndrome, Fragile X, and more.

With over a decade of global all-source intelligence experience, we follow ongoing trends in clinical research, recap important peer-reviewed medical journals, and chronicle ongoing events for innovative biotech companies with promising compounds like Anavex Life Sciences Corporation (AVXL), Cognition Therapeutics (CGTX), and more.

Anavex 2025: Partnership Discussions, FDA Application, Schizophrenia, New Indications 

Bottom-line Up Front: The last year was pivotal for Anavex Life Sciences Corporation. The most notable accomplishments include: Anavex 3-71 advancement with pharmacokinetics and initiation of a phase 2 Schizophrenia trial, additional data and publication for their landmark 48-week placebo-controlled 2b/3 Early Alzheimer's Disease trial, and subsequent topline data release for their Early Alzheimer's open-label extension trial covering up to 192 weeks of treatment at JPM Healthcare Conference 2025. In addition, the company expanded their leadership team with the hiring of  Juan Carlos Lopez-Talavera, MD, PhD as Senior Vice President, Head of Research and Development, Terrie Kellmeyer, PhD as Senior Vice President of Clinical Development, and Jeffrey Edwards, PhD as Vice President of Clinical Pharmacology and Science. These industry leaders have relevant experience at Abbvie, Bristol Myers Squibb, Roche, and Acer to name a few. Finally and most importantly, the European Medicines Agency (EMA) accepted Anavex's centralized procedure application for Alzheimer's Disease which will likely be met with regulatory decision in late 4Q 2025 with the potential for early 1Q 2026. If approved, Anavex 2-73 (Blarcamesine) would be the first available oral disease-modifying treatment, and would be accessible in all European Union (EU) states. In addition to regulatory decision from the EMA, we expect the company to advance discussion with big pharma (BP) partners, file for approval with U.S. regulators at the FDA upon publication of new guidance found within "Early Alzheimer’s Disease:  Developing Drugs for Treatment", publish phase 2 clinical data for Anavex 3-71 in Schizophrenia, and advance the long-awaited Parkinson's Disease indication. Additional catalysts possibly include further development in the Rett Syndrome and Fragile-X indications, and possibly advancements with other Anavex compounds like Anavex 1-41 and Anavex 1066.

Summary


Investment Thesis

Anavex Life Sciences Corporation (AVXL) has likely positioned itself for European approval for their lead compound, Blarcamesine, which is currently the most promising remedy for holistic neurodegenerative and neurodevelopmental diseases in development, thanks to its wide-sweeping MOA revolving around SIGMAR1 receptor (S1R/Sigma-1) & muscarinic agonist properties. In early Alzheimer's patients, Blarcamesine showed significant cognitive and global improvement for Alzheimer's patients in greater magnitude, and earlier than existing mAb therapies Aduhelm, Leqembi, and Donanemab. Additionally, the drug dramatically slowed brain atrophy and improved amyloid biomarker 42/40. Brain preservation has never been shown in a mAb trial and indicates potential preventative mechanisms. By the scientific community's own definition, Blarcamesine is a first-in-class Alzheimer's disease modifying drug, showing improvement in patient outcomes and meaningfully changing slope of disease progression. Reported in Jan 2025, Anavex maintained $132.2M in cash, which is set to finance the company for at least the next four years. Anavex maintains exceptional room for growth beyond Alzheimer's, featuring an extensive pipeline in large unmet needs, a slew of orphan disorders, a good safety profile, and an entire platform of other early-stage drugs like Anavex 3-71, Anavex 1-41, and Anavex-1066 which provide long-term growth potential. Mid-term catalysts for Anavex include an approval decision by the EMA and discussion on potential approval pathways at the FDA.


Autophagy: A Likely Driver of CNS-Wide Pathogenesis

In 1955, Christian de Duve discovered lysosomes and coined the term "autophagy" in 1963. In 1974 he was awarded the Nobel Prize in Physiology or Medicine for this cellular pioneering. Following this discovery, there was a gap of research into the mechanism until Yoshinori Ohsumi conducted autophagy experiments in yeast from the 1980s through the 1990s. From his experiments, Ohsumi uncovered autophagy-related genes and machinery driving autophagic processes which were published in 1992. This research earned him the 2016 Noble Prize in Physiology or Medicine. It was around this time that research towards autophagy took off in a meaningful way, with an 18.50% increase in autophagy-related publications year-over-year. By 2021, this figure climbed as high as 92.87% from Ohsumi's 2016 award, indicating an emphasis and scientific vigor towards describing the benefits of autophagy on lifespan, weight-loss, and CNS disorders.

Put simply, autophagy is a process in which our body degrades defective proteins or organelles in lysosomes and further recycle components of the defective proteins to be used again. Defective proteins are tagged (ubiquitinated), which marks them for degradation. This ubiquitinated cargo is then recognized by the ubiquitin-binding domain of adapter proteins and further linked to autophagosomes. The autophagosome encapsulates the ubiquitinated cargo (tagged faulty protein) and further delivers itself to the lysosome for degradation - the degradation of which is highly dependent on optimal acidification levels within the lysosome. While macroautophagy is the primary type within mammals, chaperone-mediated autophagy (CMA) has also recently been elucidated to have a critical role in the pathogenesis (the origin) of multiple human diseases including cancers and CNS disorders. It has been thoroughly investigated that CMA modulates the processing of Alzheimer's-related proteins like tau and APP (amyloid synthesis). Autophagic activity declines in age-dependent manner in both humans and animals, and it has been found that enhancing autophagic flux in mouse models improve Alzheimer's pathologies. Autophagic structures like autophagosomes and autolysosomes are accumulated in Alzheimer's patients, and autophagy-related genes are significantly upregulated in the hippocampus - suggesting a compensatory response in very early disease progression. As the disease progresses, autophagic flux becomes increasingly compromised, and when taking these facts in concert it can be deduced that dysfunctional autophagy is highly likely correlated to Alzheimer's pathogenesis.

Probably the most important papers relating to Blarcamesine's primary MOA are: Faulty autolysosome acidification in Alzheimer’s disease mouse models induces autophagic build-up of Aβ in neurons, yielding senile plaques, Calcium signaling hypothesis: A non-negligible pathogenesis in Alzheimer’s disease, and Sigma-1 Receptor Activation Induces Autophagy and Increases Proteostasis Capacity In Vitro and In Vivo

These foundational papers describe what could feasibly be believed as the origin of Alzheimer's disease. Unsurprisingly, many of the theories proposed by Alzheimer's researchers are actually tightly linked together, including mitochondrial dysfunction/oxidative stress, calcium dysfunction, neuroinflammation, tau hypothesis, the amyloid cascade, and autophagic failure. Whereas community members tend to favor one over the other, it is likely much more effective to view these theories as a complex interconnected disease cascade.

In 2022, researchers assessed five totally distinct Alzheimer's mouse models and discovered a phenomenon in which lysosomes continue taking in faulty proteins for degradation but lose the ability to actually break down these proteins. This resulted in a hoarding of defective/misfolded proteins within the lysosome, which bloated the formation of the lysosome, caused flower petal-like protrusions around the lysosome, and ultimately led to toxic leakage and mutation of the lysosome into a senile plaque. Researchers dubbed this phenomenon "PANTHOS" and noted its presence in all five mouse models well in advance of toxic amyloid plaques (~10-20 years in advance for humans). Something went wrong in the lysosomal acidification to cause these abhorrent mutations and autophagic dysfunction. It was found that in conjunction with forming PANTHOS, the mice also had markedly low levels of vATPase - a protein complex that acidifies intracellular compartments like lysosomes. We will come back to vATPase shortly.

Importantly, in Alzheimer's patients amyloid is primarily found extracellularly, and tau is found primarily intracellularly. Autophagy takes place primarily intracellularly, not where amyloid is found in abundance. During Anavex's 2b/3 Alzheimer's trial, amyloid levels in the CSF were significantly improved. While Blarcamesine's MOA is extremely robust, how could it be that amyloid improvement was caused by autophagy, if autophagy is intracellular and amyloid is extracellular? Researchers of the cornerstone PANTHOS paper address this question in the following paragraph.

"β-amyloid plaque formation in AD has commonly been considered to originate from extracellular deposition of β-amyloid derived from secreted Aβ, which then triggers secondary neuritic dystrophy and neuronal cell death. By contrast, our evidence in diverse AD models supports the opposite sequence—namely, extracellular plaques mainly evolve from intraneuronal build-up of β-amyloid within membrane tubules, forming a centralized amyloid ‘core’ within single intact PANTHOS neurons that subsequently degenerate to give rise to the classical senile plaque. This ‘inside-out’ process accords with and substantiates hypotheses from many investigators. In versions of this hypothesis, Aβ and its oligomeric species generated intracellularly within ALP compartments can gain access to the extracellular space by neurodegeneration, local membrane damage or unconventional secretion (exocytosis). Importantly, a few investigators have described intracellular membrane-enclosed amyloid fibrils in AD mouse models and, in AD brain, the frequent presence of amyloid surrounding DAPI-positive nuclei, and neuronal lysosomal hydrolase abundance within extracellular β-amyloid."

Summarizing thus far, research indicates that lysosomal acidification issues (rather de-acidifying) is possibly caused by low vATPase, which ultimately manifests senile plaques and toxic amyloid leakage - autophagy failure. It also indicates that toxic amyloid is formed intracellularly, which could theoretically be reduced or negated by improving intracellular mechanisms prophylactically or in very early disease stages.

Taking our research a step further, we introduce a second paper from 2025 covering calcium channeling and its role in Alzheimer's pathogenesis. Calcium has diverse functions including neurotransmitter release, action potential conduction, synaptic function, and neuronal growth. Virtually every process requiring synaptic activity, neuronal communication, and plasticity involves calcium messaging. The authors illustrate that vATPase and calcium are interconnected through their roles in homeostasis: "It is generally believed that vATPase in lysosomes is the prerequisite and primary regulator for maintaining the acidic environment in lysosomes and the fusion of autophagosomes and lysosomes. Down-regulated expression of vATPase in the AD brain can result in lysosomal alkalization and suppressed autophagy. However, the fusion of lysosomes and autophagosomes lacking vATPase is not disrupted, but prolonged. Fusion is only interrupted when Ca2+ [calcium] signaling and vATPase are simultaneously inhibited, and the fusion can be restored only by supplementing Ca2+. This result indicates that Ca2+ channels are more critical than vATPase in regulating lysosomal acidification". The authors then go on to describe how this process can be reversed by blocking calcium release from the endoplasmic reticulum: "Similarly, the restoration of lysosomal vATPase expression following pharmacological blockade of Ca2+ release from the ER leads to a drop in lysosomal pH, the restoration of autophagic flux, and the reduction of erroneous protein aggregation."

Concluding the segment, the authors state that calcium signaling of the endoplasmic reticulum is likely a key event in the regulation of the autophagy-lysosomal process through vATPase; acknowledging further studies are likely required to fully elucidate these relationships. 

SIGMAR1 is a chaperone protein immersed in lipid rafts of the endoplasmic reticulum where it interacts with mitochondria at the mitochondria-associated ER membrane domain (MAM). Upon activation, SIGMAR1 binds to the inositol trisphosphate receptor (IP3R), and modulates cellular calcium homeostasis. A 2015 review of SIGMAR1 stated: "Activation of the Sig-1R provides neuroprotection and is neurorestorative in cellular and animal models of neurodegenerative diseases and brain ischemia. Neuroprotection appears to be due to inhibition of cellular Ca(2+) toxicity and/or inflammation, and neurorestoration may include balancing aberrant neurotransmission or stimulation of synaptogenesis, thus remodeling brain connectivity."

SIGMAR1 has direct roles in modulating calcium signaling (increasing or decreasing dependent on need) to and from the endoplasmic reticulum - an important requirement for acidification of the autophagy-lysosomal pathway. The relationship between SIGMAR1, autophagy, and PANTHOS (early research) was first uncovered in 2013 and 2014. During those studies it was found that: "Sigmar1’s molecular role in the autophagy pathway has been made evident by studies where Sigmar1 siRNA knockdown in HEK293 and NSC34 cells led to the accumulation of numerous autophagic vacuoles often filled with non-degraded autophagic substrates and deformities of ER ultrastructure". Furthermore, mutations of the SIGMAR1 gene or SIGMAR1 knockout have both been associated with reduced ATP, increased endoplasmic reticulum stress, increased autophagosome accumulation, and apoptosis (cell death). 

In addition to regulating calcium flux, SIGMAR1 aids autophagy in more direct ways as well by stabilizing and translating LC3B mRNA for autophagsome formation. Multiple studies have now captured SIGMAR1 as a critical regulator of ATG8-family genes, as well as MAP1LC3B/LC3B and GABARAP mRNAs. Cells lacking SIGMAR1 show reduced levels of many ATG8-family proteins and impaired autophagic flux.

Blarcamesine itself was tested for autophagic response in 2019, with data published in the paper titled: Sigma-1 Receptor Activation Induces Autophagy and Increases Proteostasis Capacity In Vitro and In Vivo. The drug was tested in HeLa (human cells) as well as C. elegans (worms), which concluded with multiple compelling discoveries. In human cells, Blarcamesine significantly increased autophagic flux in a dose-dependent manner, by about 60% over controls (1 μM dose), and about 110% over controls (10 μM dose). Another SIGMAR1 agonist (PRE-084) also improved autophagic flux over controls. Additionally, it was found that SIGMAR1 expression increase by Blarcamesine intervention stimulates ULK1 activation and affects expression levels of distinct autophagy network factors. In the ULK1 assay, 1 μM dose of Blarcamesine increased expression by about 140%, and also significantly upregulated a series of autophagy-related genes including ATG12, ATG16L1, GABARAPL1, and SQSTM1. No autophagy genes were downregulated as a result of Blarcamesine.

Mechanism of Action Conclusion: Aging is the greatest predicator of Alzheimer's and greater CNS-disorders. With age come pathological changes as a result of cellular stressors, mitochondrial dysfunction (lower ATP), oxidative stress, DNA damage, inflammation, vascular diseases, and impaired proteostasis & autophagy. Cellular stress and DNA damage especially set a fragile environment prone to disease. As calcium signaling, vATPase, and autophagy-related gene function begin to wane, lysosomal bloating, leakage, and mutation into senile plaques occur with intraneuronal amyloid build-up. SIGMAR1 plays direct and indirect roles with calcium signaling, mediates calcium-vATPase cross-talk, improves faulty acidification of lysosomes, and bolsters autophagy-related gene stabilization & translation. Blarcamesine administration results in significantly improved autophagy flux and gene expression. SIGMAR1 mutation or knockout results in reduced ATP, increased cellular stress, autophagosome accumulation, and apoptosis. By all accounts, SIGMAR1 likely addresses the earliest known deficiencies that give rise to Alzheimer's pathogenesis, with autophagic dysfunction noted 10 - 20 years in advance of toxic amyloid & tau build-up. By addressing very early disease stages, SIGMAR1 agonism could potentially delay or reverse pathogenesis in a large array of CNS-disorders, as evidenced by the high-magnitude cognitive savings and brain atrophy reduction (preservation) seen in Anavex's 2b/3 Alzheimer's trial. The next segment will succinctly cover those results. 


Blarcamesine for the treatment of Early Alzheimer's Disease: Results from the ANAVEX2-73-AD-004 Phase IIB/III trial

In early Jan 2025, Anavex had the results of their landmark 48-week placebo-controlled 2b/3 Early Alzheimer's Disease trial published in The Journal of Prevention of Alzheimer's Disease. Key authors include Stephan Macfarlane, Timo Grimmer, Marwan Sabbagh, and Kun Jin - a 27 year veteran at the FDA and their former Lead Neurology Statistician; not to mention one of the key statistical reviewers for Memantine, Lecanemab, and more. Bulleted notes for the paper can be found below.

Paper Facts:


Best Paper Quotes:


Spirit of the Coast Assessments on Anavex's 2b/3 Alzheimer's Paper:


Developmental Conclusions and 2025 Look-ahead:

Spirit of the Coast Analytics will continue monitoring positive developments by Anavex Life Sciences and Cognition Therapeutics (CGTX) through 2025. SIGMAR1 and SIGMAR2 modulation appear to be highly efficacious and potentially complementary in a sweeping number of CNS-disorders. While Anavex currently holds a clear lead in Alzheimer's and Parkinson's disease, Cognition Therapeutics will potentially start a phase 3 Alzheimer's trial by the end of 2025, and make progress on a phase 3 DLB trial (dementia with lewy bodies) as well. Further phase 2 DLB data for their SHIMMER trial is expected to be released at the end of Jan 2025 (at the 8th International Lewy Body Dementia Conference) and we will make an update at that time. A company overview for Cognition can be found here, their CTAD 2024 Alzheimer's update can be found here, and their phase 2 DLB topline data can be found here.

Anavex is likely to make strides in their Anavex 3-71 Schizophrenia program, a pivotal Parkinson's disease trial featuring Blarcamesine, probable progression in the Rett syndrome and Fragile-X indications, and further elucidate open-label extension and gene data for the Alzheimer's indication. We also anticipate 2025 to be a ramp-up year for commercializing Blarcamesine in Europe.

In our opinion, Blarcamesine addresses all core aspects of early Alzheimer's pathology; cognitive loss, brain degeneration, and amyloid/protein pathological load. Cognitive loss reduction and brain atrophy slowing seen in SIGMAR1 WT patients may be strong enough to prevent MCI and early-stage Alzheimer's patients from ever reaching moderate or severe stages. With scarce commercial competition, there was never a better time for Anavex to have filed for approval in Europe (application accepted), which numerically has a larger patient population and more desirable patients (greater SIGMAR1 WT prevalence) than the United States. 

Regarding our independent marketing and partnered marketing analysis, we feel it is highly unlikely that Anavex will be able to meaningfully pursue large markets without a big pharma supporter/partner. With a partner, we have projected that at peak Alzheimer's sales in Europe only, Anavex's market cap would exceed $14B in the least desirable conditions, or be as high as $86B in most desirable conditions. Our full analysis for European and U.S. markets can be found here.

With the successful Alzheimer's data publication, centralized procedure application and acceptance by the EMA, and need to prepare for potential commercialization, we expect Anavex to place greater emphasis on partnership discussions (ongoing since at least 1Q 2022), insurers, supply chain, and market penetration throughout 2025. Updates to the novel Schizophrenia trial and Parkinson's indications specifically will drive significant value throughout the year. Finally, with the expected finalization of the FDA's early Alzheimer's guidance in 2025, we expect Anavex to have discussions with the FDA on potential approval in the United States.