Accessory component of the proton-transporting vacuolar (V)-ATPase protein pump involved in intracellular iron homeostasis. In aerobic conditions, required for intracellular iron homeostasis, thus triggering the activity of Fe(2+) prolyl hydroxylase (PHD) enzymes, and leading to HIF1A hydroxylation and subsequent proteasomal degradation. Necessary for endolysosomal acidification and lysosomal degradation (PubMed:28296633). May be involved in Golgi homeostasis (PubMed:26833330). Binds 20(S)-hydroxycholesterol (20(S)-OHC) (By similarity).

    Matrix Type

    • Tissue/Cells

    Gene Symbol

    • TMEM199

    UniProt ID

    • Q8N511

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