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ATP6V1H, also known as V-type proton ATPase subunit H, is a 483 amino acid protein that is a subunit of the V1 complex, which is responsible for acidifying eukaryotic cells in intracellular organelles. Studies on this protein have shown a relationship with the following disorders and diseases, osteopetrosis, carcinoma, cholera, colon carcinoma, arthritis, rheumatoid arthritis, neuronitis, tuberculosis, and immunodeficiency. The ATP6V1H protein has also shown an interaction with ATP6V1D, ATP6V1F, ATP6V1B2, ATP6V1E1, and ETHE1 in the oxidative phosphorylation, metabolic, phagosome, lysosome, insulin receptor recycline, and Iron uptake and transport pathways.