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MIA3
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. This protein is required for collagen VII (COL7A1) secretion by loading COL7A1 into transport carriers. It may participate in cargo loading of COL7A1 at endoplasmic reticulum exit sites by binding to COPII coat subunits Sec23/24 and guiding SH3-bound COL7A1 into a growing carrier. Does not play a role in global protein secretion and is apparently specific to COL7A1 cargo loading. However, it may participate in secretion of other proteins in cells that do not secrete COL7A1. It is also specifically required for the secretion of lipoproteins by participating in their export from the endoplasmic reticulum. Required for correct assembly of COPII coat components at endoplasmic reticulum exit sites (ERES) and for the localization of SEC16A and membrane-bound ER-resident complexes consisting of MIA2 and PREB/SEC12 to ERES. Belongs to the MIA/OTOR family. Tango1 subfamily. Broadly expressed, except in bone marrow and peripheral blood mononuclear cells. Down-regulated in melanoma tissue. 4 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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| Protein type: Cell adhesion; Endoplasmic reticulum; Membrane protein, integral; Motility/polarity/chemotaxis |
| Chromosomal Location of human Ortholog: 1q41 |
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Cellular Component:
endoplasmic reticulum exit site; endoplasmic reticulum lumen; endoplasmic reticulum membrane; intracellular membrane-bounded organelle; membrane
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Molecular Function:
cargo receptor activity; protein binding
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Biological Process:
cell migration involved in sprouting angiogenesis; cellular response to oxidised low-density lipoprotein particle stimulus; COPII-coated vesicle cargo loading; endocytosis; endoplasmic reticulum organization; endoplasmic reticulum to Golgi vesicle-mediated transport; exocytosis; lipoprotein transport; negative regulation of cell adhesion; negative regulation of cell migration; negative regulation of leukocyte cell-cell adhesion; negative regulation of lymphocyte migration; positive regulation of leukocyte migration; protein localization to endoplasmic reticulum exit site; protein secretion; protein transport; vesicle cargo loading; wound healing
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Disease: Odontochondrodysplasia 2 With Hearing Loss And Diabetes
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Reference #:
Q5JRA6
(UniProtKB)
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Alt. Names/Synonyms: ARNT; C219 reactive peptide; C219-reactive peptide; D320; FLJ21469; FLJ39207; KIAA0268; melanoma inhibitory activity family, member 3; Melanoma inhibitory activity protein 3; MIA family member 3, ER export factor; MIA SH3 domain ER export factor 3; MIA3; TANGO; TANGO1; TGO1; Transport and Golgi organization protein 1; Transport and Golgi organization protein 1 homolog; UNQ6077
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Gene Symbols: MIA3
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Molecular weight:
213,702 Da
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Basal Isoelectric point:
4.76
Predict pI for various phosphorylation states
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