J. Neurochem.(2010) 115, 297–313. Abstract Apart of its well known function of 'energetic
buffer'through the creatine/phosphocreatine/creatine kinase system allowing the …

Creatine deficiency syndromes, due to deficiencies in AGAT, GAMT (creatine synthesis
pathway) or SLC6A8 (creatine transporter), lead to complete absence or very strong …

Photoreceptors are the first‐order neurons of the visual pathway, converting light into
electrical signals. Rods and cones are the two main types of photoreceptors in the …

The human retina, which is part of the central nervous system, has exceptionally high energy
demands that requires an efficient metabolism of glucose, lipids, and amino acids …

Mutations in the creatine (Cr) transporter (CrT; Slc6a8) gene lead to absence of brain Cr and
intellectual disabilities, loss of speech, and behavioral abnormalities. To date, no mouse …

Creatine deficiency syndromes, either due to AGAT, GAMT or SLC6A8 deficiencies, lead to
a complete absence, or a very strong decrease, of creatine within the brain, as measured by …

While it was thought that most of cerebral creatine is of peripheral origin, AGAT and GAMT
are well expressed in CNS where brain cells synthesize creatine. While the creatine …

Key points• Creatine (Cr) plays an important role in muscle energy homeostasis as a
substrate in the creatine kinase phosphoryl exchange reaction, but the consequences of …

Creatine provides cells with high-energy phosphates for the rapid reconstitution of
hydrolyzed adenosine triphosphate. The eponymous creatine transporter (CRT1/SLC6A8) …

Evidence for the important physiological role of the creatine kinase (CK)–phosphocreatine
(PCr) system in energy homeostasis in sarcomeric muscle, brain, and other organs of high …