STEAP3
Function
Integral membrane protein that functions as a NADPH-dependent ferric-chelate reductase, using NADPH from one side of the membrane to reduce a Fe(3+) chelate that is bound on the other side of the membrane (PubMed:26205815). Mediates sequential transmembrane electron transfer from NADPH to FAD and onto heme, and finally to the Fe(3+) chelate (By similarity). Can also reduce Cu(2+) to Cu(1+) (By similarity). Mediates efficient transferrin-dependent iron uptake in erythroid cells (By similarity). May play a role downstream of p53/TP53 to interface apoptosis and cell cycle progression (By similarity). Indirectly involved in exosome secretion by facilitating the secretion of proteins such as TCTP (PubMed:15319436, PubMed:16651434).
Involvement in disease
Anemia, hypochromic microcytic, with iron overload 2
AHMIO2
A hematologic disease characterized by abnormal hemoglobin content in the erythrocytes which are reduced in size, severe anemia, erythropoietic hyperplasia of bone marrow, massive hepatic iron deposition, and hepatosplenomegaly.
None
The disease is caused by variants affecting the gene represented in this entry.
Post-translational modifications
Proteolytically cleaved by RHBDL4/RHBDD1. RHBDL4/RHBDD1-induced cleavage occurs at multiple sites in a glycosylation-independent manner.
Glycosylated.
Sequence Similarities
Belongs to the STEAP family.
Tissue Specificity
Expressed in adult bone marrow, placenta, liver, skeletal muscle and pancreas. Down-regulated in hepatocellular carcinoma.
Cellular localization
- Endosome membrane
- Multi-pass membrane protein
- Localizes to vesicular-like structures at the plasma membrane and around the nucleus.
Alternative names
TSAP6, STEAP3, Metalloreductase STEAP3, Dudulin-2, Six-transmembrane epithelial antigen of prostate 3, Tumor suppressor-activated pathway protein 6, pHyde, hTSAP6, hpHyde