Barry Wolf Selected Research

Biotinidase

3/2021	Biotinidase deficiency is a rare, potentially treatable cause of peripheral neuropathy with or without optic neuropathy in adults.
9/2017	Developmental window of sensorineural deafness in biotinidase-deficient mice.
1/2017	Irreversibility of Symptoms with Biotin Therapy in an Adult with Profound Biotinidase Deficiency.
12/2016	Differential gene expression during early development in brains of wildtype and biotinidase-deficient mice.
3/2016	First microdeletion involving only the biotinidase gene that can cause biotinidase deficiency: A lesson for clinical practice.
9/2011	The neurology of biotinidase deficiency.
9/2010	Analysis of mutations causing biotinidase deficiency.
7/2005	Biotinidase: its role in biotinidase deficiency and biotin metabolism.
11/2002	Localization of biotinidase in the brain: implications for its role in hearing loss in biotinidase deficiency.
6/2002	Biotin dependency due to a defect in biotin transport.

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Barry Wolf Research Topics

Disease

29	Biotinidase Deficiency (Multiple Carboxylase Deficiency, Late Onset) 03/2021 - 03/2002
4	Hearing Loss (Hearing Impairment) 09/2017 - 11/2002
2	Biotin deficiency 09/2017 - 06/2002
2	Neurocutaneous Syndromes (Phakomatoses) 02/2011 - 05/2010
1	Congenital Heart Defects (Congenital Heart Defect) 01/2021
1	Multiple Sclerosis 04/2020
1	Muscle Weakness 01/2020
1	Neurodevelopmental Disorders 01/2019
1	Acidosis 09/2017
1	Deafness (Deaf Mutism) 09/2017
1	Weight Loss (Weight Reduction) 09/2017
1	Failure to Thrive 12/2016
1	Body Weight (Weight, Body) 05/2014
1	Demyelinating Diseases (Demyelinating Disease) 09/2012
1	Hyperammonemia 02/2011
1	Optic Atrophy 02/2011
1	Paraparesis 09/2008
1	Inborn Errors Metabolism (Inborn Errors of Metabolism) 07/2003

Drug/Important Bio-Agent (IBA)

24	Biotin (Vitamin H)FDA Link 04/2020 - 03/2002
11	EnzymesIBA 01/2019 - 03/2002
10	BiotinidaseIBA 03/2021 - 06/2002
8	VitaminsIBA 09/2017 - 04/2005
1	phospholipase D1IBA 01/2021
1	hypusineIBA 01/2019
1	deoxyhypusine synthaseIBA 01/2019
1	Lysine (L-Lysine)FDA Link 12/2016
1	biocytinIBA 12/2016
1	Proteins (Proteins, Gene)FDA Link 12/2016
1	beta-hydroxyisovaleric acid (beta-hydroxy-beta-methylbutyrate)IBA 02/2011
1	AmidohydrolasesIBA 09/2005
1	Amino Acids FDA Link 09/2005
1	Complementary DNA (cDNA)IBA 11/2002
1	Ligases (Synthetase)IBA 06/2002

Therapy/Procedure

4	Therapeutics 01/2020 - 09/2012