Cure AHC funded published research

DATE AWARDED

AMOUNT

RECIPIENT

PURPOSE

RESULTS

March 2013

$1,000

Duke University

AHC Multidisciplinary Clinic

Launched Clinic, ABC & CBS Affiliates covered the story. As of 8/2017, the clinic has now seen nearly 100 AHC children and adults from around the world or be directly overseeing their care in collaboration with local care teams.

May 2013

$5,000

AIESA (Italian AHC Association

2nd ATP1A3 Symposium in Rome

Published Research Article https://www.ncbi.nlm.nih.gov/pubmed/24739246

April 2014

$20,000

Mohamad Mikati (Duke)

Mouse Model AHC

Published Research Article http://onlinelibrary.wiley.com/doi/10.1111/epi.12878/full (confirming that the mouse model very closely mirrors symptomology seen in humans, thus validating the model for drug screening)

June 2014

$10,000

David Goldstein/ Erin Heinzen (Duke)

MEA Screening for Candidate Drugs in ATP1A3 Mutations D801N & E815K

Several candidate compounds identified - referred to mouse model team for testing.

June 2014

$7,000

AHC Association of Netherlands

3rd Symposium on ATP1A3

Recruited new researchers. Official launch of the International AHC Research Consortium (IAHCRC)

October 2014

$8,000

AHC Association of Iceland

AHC Documentary "Human Timebombs"

Documentary completed http://humantimebombs.com/

January 2015

$45,000

Mohamad Mikati (Duke)

Pathophysiology of

the AHC Knock-in

Mouse Model

Results from study lead to $225,000 ($75K/year for 3 years) award from Duke Institute for Brain Sciences and poster session http://n.neurology.org/content/86/16_Supplement/S52.006 (E815K Mouse model also viable match to human AHC of same mutation origin)

July 2015

$50,000

N/A

Family Gathering and hosted 4th ATP1A3 Symposium in Bethesda, MD

20 families attended, streamed via GoToMeeting to another 25, got attention of FDA and NIH officials, added new researchers, new collaborative ideas generated

September 2015

$59,400

Duke University

Clinical Research Coordinator

*Increased the capacity of the Duke Multidisciplinary Clinic. *Guidelines for treating AHC Symptoms (dramatically improving quality of life).* Several published papers and presentations 1. http://www.neurology.org/content/86/16_Supplement/I15.001 (reporting preliminary findings that VNS can potentially help AHC patients with intractable seizures) 2. https://www.ncbi.nlm.nih.gov/pubmed/28337648 (demonstrating that motor speech deficiency is a hallmark of the disease. This helps validate the need for speech therapy (for insurance coverage) and can serve as a biomarker to test efficacy of target compounds) 3. https://link.springer.com/article/10.1007/s11940-017-0444-7 (this is a comprehensive tool for diagnosing AHC and improving treatment approaches) 4. http://www.neurology.org/content/88/16_Supplement/P3.198.short (identified characteristics of status epilepticus in AHC, to help guide treatment and understand potential impact to future health)

November 2015

$75,000

Erin Heinzen/ David Goldstein (Columbia)

Identify neuronal properties of AHC mutant mouse neurons at baseline conditions, investigate the response mutant neurons to biologically relevant stimuli, ascertain the effect of pharmacological intervention on mutant neuronal networks

Publication pending

July 2016

$41,000

Mohamad Mikati (Duke)

Catastrophic Outcomes in the AHC Knock-in Mouse Models D801N and E815K for AHC

Work in progress

October 2016

$25,000

Erin Heinsen/ David Goldstein (Columbia)

Phase 2 of Optogenetics Study

Publication pending

December 2016

$55,000

Mohamad Mikati (Duke)

mRNA Study (strategic development of novel compounds to treat disease at molecular level

Work in progress, funding still needed

December 2016

$50,000

Mohamad Mikati (Duke)

ATP1A2 Mouse Model

Work in progress, funding still needed

February 2017

$15,000

IAHCRC (Duke University)

OBSERV-AHC (Natural History Project)

Work in progress, funding still needed

March 2017

$6,000

IAHCRC

IAHCRC Cloud Platform

Joint efforts with AHC Associations of UK, Iceland & Netherlands

July 2017

$45,000

N/A

Family Gathering in Boston, MA

Provided stipends to offset costs to 25 families

July 2017

$33,000

Karin Lykke-Hartmann (Aarhus University, Denmark)

To analyze the underlying mechanism that cause the cognitive impairment

Work in progress