Braissant · Journal of inherited metabolic disease 2012 · narrative review · n=?

Creatine and guanidinoacetate transport at blood-brain and blood-cerebrospinal fluid barriers.

Cited 111 times in the scientific literature.

Level 5 - mechanism / opinion, no new human data

Narrative review synthesizing mechanistic transport data and clinical deficiency syndromes without systematic methodology.

PubMed 22252611 · doi:10.1007/s10545-011-9433-2 · record verified 2026-08-26

What was done

The authors synthesized mechanistic and clinical literature regarding creatine and guanidinoacetate transport across the blood-brain barrier (BBB) and blood-cerebrospinal fluid barrier (BCSFB), incorporating data from AGAT-, GAMT-, and SLC6A8-deficient patients as well as cellular expression studies.

What was found

The abstract reports no numerical metrics. It summarizes that the creatine transporter SLC6A8 is expressed in microcapillary endothelial cells but absent from surrounding astrocytes, restricting peripheral creatine permeability and necessitating endogenous central nervous system (CNS) synthesis via AGAT and GAMT. Under physiological conditions, peripheral guanidinoacetate is not transported into the CNS across the BBB (except under GAMT deficiency), but undergoes net efflux from cerebrospinal fluid to blood across the BCSFB, primarily mediated by the taurine transporter TauT.

Why it matters

These transport dynamics explain why peripheral creatine supplementation cannot effectively treat SLC6A8 creatine transporter deficiency and identify the mechanisms governing guanidinoacetate clearance from the CNS.

Limits

The paper is a narrative review without systematic search protocols, quantitative data extraction, or meta-analysis. Findings rely heavily on mechanistic reasoning and rare genetic deficiency syndromes, with no quantitative human kinetic data reported in the abstract.

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