ATP6V0D1

This gene encodes a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of eukaryotic intracellular organelles. V-ATPase dependent organelle acidification is necessary for such intracellular processes as protein sorting, zymogen activation, receptor-mediated endocytosis, and synaptic vesicle proton gradient generation. V-ATPase is composed of a cytosolic V1 domain and a transmembrane V0 domain. The V1 domain consists of three A and three B subunits, two G subunits plus the C, D, E, F, and H subunits. The V1 domain contains the ATP catalytic site. The V0 domain consists of five different subunits: a, c, c', c'', and d. Additional isoforms of many of the V1 and V0 subunit proteins are encoded by multiple genes or alternatively spliced transcript variants. This encoded protein is known as the D subunit and is found ubiquitously. [provided by RefSeq]

Full Name

ATPase, H+ transporting, lysosomal 38kDa, V0 subunit d1

Function

Subunit of the integral membrane V0 complex of the lysosomal proton-transporting V-type ATPase (v-ATPase) (PubMed:28296633, PubMed:30374053).
V-ATPase is responsible for acidifying a variety of intracellular compartments in eukaryotic cells, thus providing most of the energy required for transport processes in the vacuolar system (PubMed:30374053).
May play a role in coupling of proton transport and ATP hydrolysis (By similarity).
In aerobic conditions, involved in intracellular iron homeostasis, thus triggering the activity of Fe2+ prolyl hydroxylase (PHD) enzymes, and leading to HIF1A hydroxylation and subsequent proteasomal degradation (PubMed:28296633).
May play a role in cilium biogenesis through regulation of the transport and the localization of proteins to the cilium (By similarity).

Biological Process

Cellular iron ion homeostasis Source: UniProtKB
Cellular response to amino acid starvation Source: Reactome
Cellular response to increased oxygen levels Source: UniProtKB
Cilium assembly Source: UniProtKB
Insulin receptor signaling pathway Source: Reactome
Ion transmembrane transport Source: Reactome
IRE1-mediated unfolded protein response Source: Reactome
Phagosome acidification Source: Reactome
Proton transmembrane transport Source: UniProtKB
Regulation of macroautophagy Source: ParkinsonsUK-UCL
Transferrin transport Source: Reactome
Vacuolar acidification Source: GO_Central
Vacuolar transport Source: GO_Central

Cellular Location

Lysosome membrane; Membrane. Localizes to centrosome and the base of the cilium.