Immupharma Regulatory News (IMM)

FOR IMMEDIATE RELEASE 14 JUNE 2018

This announcement contains inside information for the purposes of Article 7 of Regulation (EU) 596/2014.

ImmuPharma PLC

("ImmuPharma" or the "Company")

Positive results of P140 (Lupuzor™ or Forigerimod) published in the Journal of Autoimmunity

in a model of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

The T cell modulator P140 significantly reduced the disease in the murine preclinical model

Data provides evidence for the expansion of P140 in an autoimmune indication with

'Orphan Drug Designation'

ImmuPharma PLC (LSE:IMM), ("ImmuPharma" or the "Company"), the specialist drug discovery and development company, is delighted to provide insight into its positive results from pre-clinical studies of the P140 peptide for Chronic Inflammatory Demyelinating Polyneuropathy ('CIDP'), a relapsing-remitting autoimmune-mediated inflammatory disease of the peripheral nervous system for which specifically-targeted therapies are still lacking.

The results have been published in the 'Journal of Autoimmunity' entitled: "An autophagy-targeting peptide to treat chronic inflammatory demyelinating polyneuropathies". This paper is available to review on line at the Journal of Autoimmunity: https://doi.org/10.1016/j.jaut.2018.05.009

This study was headed by Professor Sylviane Muller, inventor of P140/Lupuzor™, ImmuPharma's lead compound for Lupus, a potentially life threatening auto-immune disease.

It has already been demonstrated by a number of presentations by the Company that the mechanism of action of P140/Lupuzor™ can potentially be applied to a number of other auto-immune diseases in addition to Lupus.

Key highlights of the CIDP pre-clinical study include:

· Macro-autophagy and chaperone-mediated autophagy (CMA), are significantly altered in non-neuronal cells of the peripheral nervous system

· P140/Lupuzor™, known to target CMA and successfully used in pathological settings where CMA markers are overexpressed, considerably ameliorated the clinical and biological course of the disease in the CIDP model used

· P140 displayed prophylactic and therapeutic effects, both in terms of disease intensity and chronicity, and preserved sciatic nerves from disease-related damages

· The findings uncover new disrupted molecular pathways in the model and provide a proof-of-concept that targeting CMA might represent a promising therapeutic strategy for treating inflammatory neuropathies for which no disease-specific treatment is currently available.

Commenting on this announcement, Professor Sylviane Muller said:

"Over the last couple of years we have provided further evidence of the role the P140 peptide can take in the potential treatment of other autoimmune diseases in addition to Lupus. We are delighted that these results demonstrate positive pre-clinical data in a murine model of CIDP, which has no available disease-specific treatment. We look forward to providing a further update on the progress of our CIDP programme in due course."

Notes to Editors

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare disorder of the peripheralnerves characterized by gradually increasing sensory loss and weakness associated with loss of reflexes.

The number of new cases per year of CIDP is about 1-2 per 100,000 people, but as the disease can be present in a person for years prior to diagnosis, the prevalence reflecting the accumulation of cases over time may be as high as 9 per 100,000 in some areas.

CIDP is caused by damage to the covering of the nerves, called myelin. It can start at any age and is more frequent in men than women.

CIDP is not self-limiting or spontaneous. Left untreated, 30% of CIDP patients will progress to wheelchair dependence. Early recognition and proper treatment can avoid a significant amount of disability.

To date treatments of CIDP (corticosteroids and intravenous immunoglobulin ('IVIg')) reduce relapse frequency but are not curative. Furthermore, corticosteroids may induce severe side effects over a long time treatment period and IVIg is very expensive.

ImmuPharma PLC 

ImmuPharma is a pharmaceutical development company listed since 2006 on AIM of the London Stock Exchange (AIM: IMM), focusing on developing novel medicines with high sales potential in specialist markets with serious unmet need. ImmuPharma is led by a commercially focused Board and management team with extensive experience.

Lupuzor™

Lupuzor™ (also referred to as Forigerimod, or P140) is ImmuPharma's lead compound and a potential treatment for lupus (or Systemic Lupus Erythematosus), a chronic, potentially life-threatening auto-immune disease. Lupuzor™ has a novel mechanism of action aimed at modulating the body's immune system so that it does not attack healthy cells, and avoids causing adverse side effects. It has the potential to halt the progression of the disease in a substantial proportion of patients.

Lupuzor™ was granted Fast Track status by the US FDA and approval to start Phase III under Special Protocol Assessment (SPA) comprising of two phase III trials. This SPA was subsequently amended due to its strong safety profile to allow for a reduced number of patients in the pivotal Phase III trial thereby reducing the projected cost and time of development considerably.

The recently completed pivotal Phase III clinical trial was entitled "A 52-Week, Randomized, Double-Blind, Parallel-Group, Placebo-Controlled Study to Evaluate the Efficacy and Safety of a 200-mcg Dose of IPP-201101 Plus Standard of Care in Patients With Systemic Lupus Erythematosus".

Commercial Opportunity

There are an estimated five million people globally suffering from Lupus, with approximately 1.5 million patients in the US, Europe and Japan (Source: Lupus Foundation of America). Current 'standard of care' treatments, including steroids and immunosuppressants, can potentially have either serious side effects for patients or limited effectiveness, with over 60% of patients not adequately treated.

P140/Forigerimod in other indications

ImmuPharma together with Professor Sylviane Muller, Lupuzor's inventor, have presented new evidence supporting Lupuzor's™ Forigerimod / P140 peptide activity in several other major auto-immune disease indications outside of Lupus. In particular, the peptide appears to have general effects against chronic inflammatory indications and pre-clinical evidence supports the molecule's use in: Neuropsychiatric lupus (NPSLE); Gougerot-Sjögren syndrome (GSS); Chronic Inflammatory Demyelinating Polyneuropathy (CIDP); Arthritis; Crohn's Disease and Asthma.

Oncology and Ophthalmology

ImmuPharma's second most advanced pipeline programme, IPP-204106, is a potential treatment for various cancers and acts by modulating angiogenesis and proliferation. The programme involves the development of synthetic peptides, Nucants, which target certain nuclear proteins such as nucleolin and nucleophosmin on the surface of cells, with very high affinity and selectivity. Nucleolin is a protein which controls critical pathways within the cell. The protein is over-expressed at the surface of dividing cells which makes its binding with Nucants very attractive because of its potential selectivity - this is of particular importance in tumour targeting. We are also investigating its use in age-related macular degeneration where it has demonstrated positive preclinical efficacy results, diabetic retinopathy and other ophthalmological indications.

Metabolism and Diabetes

ImmuPharma's subsidiary 'Ureka' has initiated the development of a novel and innovative peptide technology platform through the collaboration with CNRS, gaining access to pioneering research centred on novel peptide drugs at the University of Bordeaux and the Institut Européen de Chimie et Biologie (IECB). Jointly, ImmuPharma and CNRS have filed a new co-owned patent controlling this breakthrough peptide technology. Ureka's current focus is in Diabetes Type II (GLP-1 analogues - once a month administration); Non-Alcoholic Steato-Hepatitis (NASH) and there is also potential in cancer treatment (protein/protein interaction; P53 gene).