Gamma-hydroxybutyrate (GHB) content in hair samples correlates negatively with age in succinic semialdehyde dehydrogenase deficiency

S. S. Johansen, X. Wang, D. Sejer Pedersen, P. L. Pearl, J. B. Roullet, G. R. Ainslie, K. R. Vogel, K. M. Gibson

Research output: Chapter in Book/Report/Conference proceedingChapter

8 Scopus citations


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).

Original languageEnglish (US)
Title of host publicationJIMD Reports
Number of pages6
StatePublished - 2017

Publication series

NameJIMD Reports
ISSN (Print)2192-8304
ISSN (Electronic)2192-8312


  • GABA metabolism
  • Gamma-hydroxybutyrate (GHB)
  • Hair analysis
  • Succinic semialdehyde dehydrogenase (SSADH)
  • Succinic semialdehyde dehydrogenase deficiency (SSADHD)
  • Tandem mass spectrometry

ASJC Scopus subject areas

  • Internal Medicine
  • Endocrinology, Diabetes and Metabolism
  • Biochemistry, Genetics and Molecular Biology (miscellaneous)


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