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Balanced Objects

Research Opportunities

Current Projects as of 5/2024

Drug Repurposing

Perlara is helping us search for drug therapies

In late 2023, we raised money to complete a drug repurposing screen that tests FDA approved compounds on yeast that has ADSLD. Each of the 'hits' that the screen provides will give us a new molecule to research and may help deepen our understanding of the mechanism by which the deficiency causes disease. 

ADSLD in the Newborn Screen

A team at Baylor is investigating if ADSLD has a blood biomarker

As we search for therapies for the disorder, we know early diagnosis is key to improve patient outcomes. That's why we're participating in a study that seeks to learn if ADSLD and 19 other rare disorders can be detected in blood samples and added to a Universal Newborn Screen.

Natural History Study

Dr. Oleg Shchelochkov from the NIH is accumulating knowledge

A Natural History Study observes a disease over time in order to understand the lived experience of the patient and the progression of disease. Such a study will contribute a vast amount of knowledge and will serve as a baseline for further research going forward. Families in the U.S. are encouraged to participate!

Rare-X Data Collection

Our stories become data, and data is powerful

We partner with Rare-X to collect information from patient surveys that illuminate trends in ADSLD. This data can be shared with research partners, with doctors, and even with schools to improve care for those living with ADSLD. 

Our Scientific Advisory Board

Journal Articles by Decade

2020 - Present

 

Clinical and molecular characterization of patients with adenylosuccinate lyase deficiency


ADSL Deficiency – The Lesser-Known Metabolic Epilepsy in Infancy


Adenylosuccinate lyase deficiency affects neurobehavior via perturbations to tyramine signaling in Caenorhabditis elegans


Clinical, biochemical and molecular genetic correlations in adenylosuccinate lyase deficiency


Child with adenylosuccinate lyase deficiency caused by a novel complex heterozygous mutation in the ADSL gene: A case report


Pathway-specific effects of ADSL deficiency on neurodevelopment


Adenylosuccinate lyase deficiency affects neurobehavior via perturbations to tyramine signaling in Caenorhabditis elegans


Electroclinical features and phenotypic differences in adenylosuccinate lyase deficiency: Long-term follow-up of seven patients from four families and appraisal of the literature


Adenylosuccinate lyase Deficiency (ADSL) and Report the First Case from Iran
 


2010-2019

Adenylosuccinate lyase deficiency

Novel proton MR spectroscopy findings in adenylosuccinate lyase deficiency


Diagnosis of adenylosuccinate lyase deficiency by metabolomic profiling in plasma reveals a phenotypic spectrum

Novel mutations in ADSL for Adenylosuccinate Lyase Deficiency identified by the combination of Trio-WES and constantly updated guidelines

A mild form of adenylosuccinate lyase deficiency in absence of typical brain MRI features diagnosed by whole exome sequencing

Structural and Biochemical Characterization of Human Adenylosuccinate Lyase (ADSL) and the R303C ADSL Deficiency-Associated Mutation

Novel features in the evolution of adenylosuccinate lyase deficiency

Neurologic Presentation, Diagnostics, and Therapeutic Insights in a Severe Case of Adenylosuccinate Lyase Deficiency



2000-2009

 

Misleading behavioural phenotype with adenylosuccinate lyase deficiency

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Clinical, biochemical and molecular genetic correlations in adenylosuccinate lyase deficiency

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Lethal Fetal and Early Neonatal Presentation of Adenylosuccinate Lyase Deficiency: Observation of 6 Patients in 4 Families

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Adenylosuccinate lyase deficiency: The first identified polish patient

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Human adenylosuccinate lyase (ADSL), cloning and characterization of full-length cDNA and its isoform, gene structure and molecular basis for ADSL deficiency in six patients

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Before 2000

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​Two novel mutant human adenylosuccinate lyases (ASLs) associated with autism and characterization of the equivalent mutant Bacillus subtilis ASL

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