title: Gamma-Hydroxybutyrate (GHB) Content in Hair Samples Correlates Negatively with Age in Succinic Semialdehyde Dehydrogenase Deficiency.
abstract: Gamma-hydroxybutyrate (GHB) is a drug of abuse, an approved therapeutic for narcolepsy, an agent employed for facilitation of sexual assault, as well as a biomarker of succinic semialdehyde dehydrogenase deficiency (SSADHD). Our laboratory seeks to identify surrogate biomarkers in SSADHD that can shed light on the developmental course of this neurometabolic disease. Since GHB may be quantified in hair as a potential surrogate to identify victims of drug-related assault, we have opted to examine its level in SSADHD. We quantified GHB in hair derived from ten patients with SSADHD, and documented a significant negative age correlation. These findings are consistent with recent results in patient biological fluids, including plasma and red blood cells. These findings may provide additional insight into the developmental course of SSADHD (Jansen et al., J Inherit Metab Dis 39:795-800, 2016).
authors.affiliation: Section of Forensic Chemistry, Department of Forensic Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
authors.forename: S S
authors.initials: SS
authors.lastname: Johansen
authors.affiliation: Section of Forensic Chemistry, Department of Forensic Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
authors.forename: X
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Drug Design and Pharmacology, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark.
authors.forename: D
authors.initials: D
authors.lastname: Sejer Pedersen
authors.affiliation: Department of Neurology, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.
authors.forename: P L
authors.initials: PL
authors.lastname: Pearl
authors.affiliation: Section of Experimental and Systems Pharmacology, College of Pharmacy, Washington State University, Pharmaceutical and Biomedical Sciences Building, Room 347, Health Sciences Campus, 412 E. Spokane Falls Blvd., Spokane, WA, 99214, USA.
authors.forename: J-B
authors.initials: JB
authors.lastname: Roullet
authors.affiliation: Section of Experimental and Systems Pharmacology, College of Pharmacy, Washington State University, Pharmaceutical and Biomedical Sciences Building, Room 347, Health Sciences Campus, 412 E. Spokane Falls Blvd., Spokane, WA, 99214, USA.
authors.forename: G R
authors.initials: GR
authors.lastname: Ainslie
authors.affiliation: Section of Experimental and Systems Pharmacology, College of Pharmacy, Washington State University, Pharmaceutical and Biomedical Sciences Building, Room 347, Health Sciences Campus, 412 E. Spokane Falls Blvd., Spokane, WA, 99214, USA.
authors.forename: K R
authors.initials: KR
authors.lastname: Vogel
authors.affiliation: Section of Experimental and Systems Pharmacology, College of Pharmacy, Washington State University, Pharmaceutical and Biomedical Sciences Building, Room 347, Health Sciences Campus, 412 E. Spokane Falls Blvd., Spokane, WA, 99214, USA. mike.gibson@wsu.edu.
authors.forename: K M
authors.initials: KM
authors.lastname: Gibson
keywords: GABA metabolism; Gamma-hydroxybutyrate (GHB); Hair analysis; Succinic semialdehyde dehydrogenase (SSADH); Succinic semialdehyde dehydrogenase deficiency (SSADHD); Tandem mass spectrometry