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Histidine decarboxylase deficiency causes tourette syndrome: parallel findings in humans and mice



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What the research more correctly shows is that histidine deficiency in general is one cause of Tourette's.

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Neuron. 2014 Jan 8;81(1):77-90. doi: 10.1016/j.neuron.2013.10.052.

Histidine decarboxylase deficiency causes tourette syndrome: parallel findings in humans and mice.

Castellan Baldan L1, Williams KA2, Gallezot JD3, Pogorelov V1, Rapanelli M1, Crowley M4, Anderson GM5, Loring E6, Gorczyca R7, Billingslea E1, Wasylink S1, Panza KE4, Ercan-Sencicek AG8, Krusong K9, Leventhal BL10, Ohtsu H11, Bloch MH2, Hughes ZA7, Krystal JH1, Mayes L12, de Araujo I13, Ding YS3, State MW14, Pittenger C15.


Tourette syndrome (TS) is characterized by tics, sensorimotor gating deficiencies, and abnormalities of cortico-basal ganglia circuits.

A mutation in histidine decarboxylase (Hdc), the key enzyme for the biosynthesis of histamine (HA), has been implicated as a rare genetic cause. Hdc knockout mice exhibited potentiated tic-like stereotypies, recapitulating core phenomenology of TS; these were mitigated by the dopamine (DA) D2 antagonist haloperidol, a proven pharmacotherapy, and by HA infusion into the brain.

Prepulse inhibition was impaired in both mice and humans carrying Hdc mutations.

HA infusion reduced striatal DA levels; in Hdc knockout mice, striatal DA was increased and the DA-regulated immediate early gene Fos was upregulated. DA D2/D3 receptor binding was altered both in mice and in humans carrying the Hdc mutation.

These data confirm histidine decarboxylase deficiency as a rare cause of TS and identify HA-DA interactions in the basal ganglia as an important locus of pathology.

PMID: 24411733

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