Mitochondrial thiamine pyrophosphate carrier

Definition:

Mitochondrial transporter mediating uptake of thiamine diphosphate into mitochondria. It is not clear if the antiporter activity is affected by the membrane potential or by the proton electrochemical gradient.

References:

[1]. Subrata Sabui, et al. Structure-function characterization of the human mitochondrial thiamin pyrophosphate transporter (hMTPPT; SLC25A19): Important roles for Ile(33), Ser(34), Asp(37), His(137) and Lys(291). Biochim Biophys Acta. 2016 Aug;1858(8):1883-90. [Content Brief]

[2]. Jonghoon Kang, et al. The evidence that the DNC (SLC25A19) is not the mitochondrial deoxyribonucleotide carrier. Mitochondrion. 2008 Mar;8(2):103-8. [Content Brief]

[3]. Yuanying Chen, et al. Identification and functional analysis of novel SLC25A19 variants causing thiamine metabolism dysfunction syndrome 4. Orphanet J Rare Dis. 2021 Sep 29;16(1):403. [Content Brief]

[4]. Marjorie J Lindhurst, et al. Knockout of Slc25a19 causes mitochondrial thiamine pyrophosphate depletion, embryonic lethality, CNS malformations, and anemia. Proc Natl Acad Sci U S A. 2006 Oct 24;103(43):15927-32. [Content Brief]

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