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Protein transport protein Sec23A

Domain

The Gelsolin-like repeat mediates interaction with proteins containing PPP motifs that include MIA2, MIA3 but also SEC31A. These interactions are probably competitive.

Function

Component of the coat protein complex II (COPII) which promotes the formation of transport vesicles from the endoplasmic reticulum (ER). The coat has two main functions, the physical deformation of the endoplasmic reticulum membrane into vesicles and the selection of cargo molecules for their transport to the Golgi complex. Required for the translocation of insulin-induced glucose transporter SLC2A4/GLUT4 to the cell membrane (By similarity).

Involvement in disease

Craniolenticulosutural dysplasia

CLSD

Autosomal recessive syndrome characterized by late-closing fontanels, sutural cataracts, facial dysmorphisms and skeletal defects.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence Similarities

Belongs to the SEC23/SEC24 family. SEC23 subfamily.

Tissue Specificity

Ubiquitously expressed.

Cellular localization

Alternative names

Protein transport protein Sec23A, hSec23A, SEC23-related protein A, SEC23A

swissprot:Q15436 omim:610511 entrezGene:10484