TCDB is operated by the Saier Lab Bioinformatics Group
TCIDNameDomainKingdom/PhylumProtein(s)
9.B.113.1.1









The endoplasmic reticulum (ER) membrane melanoma inhibitory activity, member3 (Mia 3; TANGO1) (1930 aas; 3 TMSs), one N-terminal and two together near the C-terminus. It is required for collagen and protein secretion in mice (Wilson et al., 2011; Ishikawa et al. 2016). (It resembles 1.I.1.1.1 with the latter showing many repeats that are homologous to residues 1230-1630 in Mia3).

Eukaryota
Metazoa
Mia3 of Mus musculus (Q8BI84)
9.B.113.1.2









Transport and golgic organization protein 1, Mia1 or TANGO1, of 1907 aas and 3 potential TMSs, one N-terminal and two closely positioned at about residue 1200.  It plays a role in the transport of cargos that are too large to fit into COPII-coated vesicles and require specific mechanisms to be incorporated into membrane-bound carriers and exported from the endoplasmic reticulum (ER). This protein is required for collagen VII (COL7A1) secretion by loading COL7A1 into transport carriers. It may participate in cargo loading of COL7A1 at ER exit sites by binding to COPII coat subunits Sec23/24 and guiding SH3-bound COL7A1 into a growing carrier. It does not play a role in global protein secretion and is apparently specific to COL7A1 cargo loading. It is required for the secretion of lipoproteins by participating in their export from the ER (Santos et al. 2016). It is localised at ER exit sites, where it binds bulky cargo within the ER lumen and recruits membranes from the ER Golgi intermediate compartment to create an exit route for their export. Lekszas et al. 2020 reported the first TANGO1-associated syndrome in humans. A synonymous substitution results in exon eight skipping, ultimately leading to a truncated TANGO1 protein,and it causes disease that involves severe dentinogenesis imperfecta, short stature, various skeletal abnormalities, insulin-dependent diabetes mellitus, sensorineural hearing loss, and mild intellectual disability. Functional studies revealed that the corresponding truncated TANGO1 protein is dispersed in the ER, and its expression in cells with intact endogenous TANGO1 impairs cellular collagen I secretion (Lekszas et al. 2020).

 

Eukaryota
Metazoa
Mia1 of Homo sapiens
9.B.113.1.3









Transport and Golgi organization protein, TANGO, of 1430 aas and probably 3 TMSs, one N-terminal and two in the middle of the protein. It is required for protein secretion (Bard et al. 2006) and may participate in cargo loading by binding to COPII coat subunits and guiding SH3-bound proteins into a growing carrier (Bard et al. 2006). At basal transitional ER sites in follicle epithelial cells, it mediates the exit of basal membrane proteins such as Vkg, LanB1 and Trol, from the endoplasmic reticulum (ER) to basal Golgi clusters (Lerner et al. 2013).  Homologues may exist in all eukaryotes and function in similar capacities (Elliott et al. 2020; Saito and Maeda 2019).

Eukaryota
Metazoa
TANGO of Drosophila melanogaster
9.B.113.1.4









Mia2 or cTAGE5 of 1412 aas and 3 TMSs, one N-terminal and two closely together in the middle of the protein. It plays a role in the transport of cargos that are too large to fit into COPII-coated vesicles and require specific mechanisms to be incorporated into membrane-bound carriers and exported from the endoplasmic reticulum (Santos et al. 2016).

Eukaryota
Opisthokonta
Mia2 of Homo sapiens