Reuben Matalon Selected Research

aspartoacylase

12/2020	Cell-Based Therapy for Canavan Disease Using Human iPSC-Derived NPCs and OPCs.
1/2017	Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease.
1/2016	rAAV Gene Therapy in a Canavan's Disease Mouse Model Reveals Immune Impairments and an Extended Pathology Beyond the Central Nervous System.
2/2011	Modification of aspartoacylase for potential use in enzyme replacement therapy for the treatment of Canavan disease.
11/2010	Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.
11/2009	Lack of aspartoacylase activity disrupts survival and differentiation of neural progenitors and oligodendrocytes in a mouse model of Canavan disease.
4/2007	Absence-like and tonic seizures in aspartoacylase/attractin double-mutant mice.
7/2006	Upregulation of aspartoacylase activity in the duodenum of obesity induced diabetes mouse: implications on diabetic neuropathy.
1/2006	Canavan disease: studies on the knockout mouse.
7/2005	Aspartoacylase gene knockout in the mouse: impact on reproduction.

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Reuben Matalon Research Topics

Disease

15	Canavan Disease (Leukodystrophy, Spongiform) 06/2022 - 05/2003
12	Phenylketonurias (Phenylketonuria) 01/2007 - 12/2003
3	Neurodegenerative Diseases (Neurodegenerative Disease) 12/2013 - 10/2004
3	Otitis Media 05/2011 - 12/2006
3	Maternal Phenylketonuria 12/2003 - 12/2003
2	Megalencephaly 07/2015 - 11/2003
2	Respiratory Tract Infections (Respiratory Tract Infection) 05/2011 - 07/2009
2	Otitis 05/2011 - 07/2009
2	Neoplasms (Cancer) 12/2006 - 01/2004
1	Demyelinating Diseases (Demyelinating Disease) 12/2020
1	Language Development Disorders (Semantic-Pragmatic Disorder) 07/2015
1	Persistent Mullerian duct syndrome 04/2015
1	Congenital Disorders of Glycosylation 02/2014
1	Cleidocranial Dysplasia (Cleidocranial Dysostosis) 07/2011
1	Nervous System Diseases (Neurological Disorders) 02/2011
1	Gliosis 11/2010
1	Maple Syrup Urine Disease 08/2010
1	Seizures (Absence Seizure) 04/2007
1	Necrosis 12/2006
1	Glycogen Storage Disease Type II (Pompe's Disease) 11/2006
1	Glycogen Storage Disease (Glycogenosis) 11/2006
1	Obesity 07/2006
1	Diabetic Neuropathies (Diabetic Neuropathy) 07/2006
1	Infections 01/2004
1	Virus Diseases (Viral Diseases) 01/2004
1	Disease Progression 11/2003
1	Digitorenocerebral Syndrome 12/2002
1	Deafness (Deaf Mutism) 12/2002

Drug/Important Bio-Agent (IBA)

13	aspartoacylaseIBA 12/2020 - 05/2003
8	N-acetylaspartate (N-acetyl aspartate)IBA 12/2020 - 09/2003
8	EnzymesIBA 02/2011 - 11/2004
6	Phenylalanine (L-Phenylalanine)FDA Link 01/2007 - 12/2003
5	Phenylalanine Hydroxylase (Phenylalanine 4 Monooxygenase)IBA 06/2005 - 12/2003
4	sapropterin (tetrahydrobiopterin)FDA Link 01/2007 - 11/2004
4	Amino Acids FDA Link 01/2007 - 08/2004
3	CytokinesIBA 05/2011 - 12/2006
2	InterleukinsIBA 05/2011 - 07/2009
2	SmokeIBA 05/2011 - 12/2006
2	AcidsIBA 08/2010 - 11/2006
2	OrexinsIBA 04/2004 - 12/2003
1	Phosphotransferases (Kinase)IBA 07/2015
1	Hormones (Hormone)IBA 04/2015
1	Glycoproteins (Glycoprotein)IBA 02/2014
1	Transcription Factors (Transcription Factor)IBA 07/2011
1	Pneumococcal Vaccines (Pneumococcal Polysaccharide Vaccine)IBA 05/2011
1	Anti-Bacterial Agents (Antibiotics)IBA 05/2011
1	Staphylococcal Protein A (Protein A)IBA 11/2010
1	Histones (Histone)IBA 11/2010
1	ChromatinIBA 11/2010
1	Branched-Chain Amino AcidsIBA 08/2010
1	Oxidoreductases (Dehydrogenase)IBA 08/2010
1	DNA (Deoxyribonucleic Acid)IBA 07/2009
1	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 07/2009
1	Interleukin-1beta (Interleukin 1 beta)IBA 12/2006
1	alpha-Glucosidases (Acid Maltase)IBA 11/2006
1	Hyaluronoglucosaminidase (Hyaluronidase)FDA Link 11/2006
1	GlucosidasesIBA 11/2006
1	trabecular meshwork-induced glucocorticoid response proteinIBA 07/2005
1	calpastatinIBA 06/2005
1	Acetates (Acetic Acid Esters)FDA Link 04/2005
1	Aspartic Acid (Aspartate)FDA Link 04/2005
1	NeuropeptidesIBA 08/2004
1	Glutamate Carboxypeptidase II (N-Acetylaspartylglutamate Peptidase)IBA 08/2004
1	isospaglumic acid (NAAGA)IBA 08/2004
1	BiopterinsIBA 01/2004
1	Interleukin-10 (Interleukin 10)IBA 01/2004
1	Non-Pku Mild HyperphenylalaninemiaIBA 12/2003
1	CreatinineIBA 11/2003
1	ProteomeIBA 09/2003
1	Glutamic Acid (Glutamate)FDA Link 08/2003
1	Serine Proteinase Inhibitors (Serine Protease Inhibitors)IBA 08/2003
1	gamma-Aminobutyric Acid (GABA)IBA 08/2003
1	Amino Acid Transport System X-AG (Glutamate Transporter)IBA 08/2003
1	Biomarkers (Surrogate Marker)IBA 12/2002
1	Ketoglutaric Acids (Ketoglutaric Acid)IBA 12/2002
1	Ketoglutarate Dehydrogenase Complex (Oxoglutarate Dehydrogenase)IBA 12/2002

Therapy/Procedure

3	Therapeutics 06/2022 - 12/2013
2	Enzyme Replacement Therapy 02/2011 - 11/2006
1	Middle Ear Ventilation (Tympanostomy) 12/2006
1	Oral Administration 11/2004
1	Transplantation 10/2004
1	Enzyme Therapy 08/2004