ID:SC23B_HUMAN DESCRIPTION: RecName: Full=Protein transport protein Sec23B; AltName: Full=SEC23-related protein B; FUNCTION: Component of the COPII coat, that covers ER-derived vesicles involved in transport from the endoplasmic reticulum to the Golgi apparatus. COPII acts in the cytoplasm to promote the transport of secretory, plasma membrane, and vacuolar proteins from the endoplasmic reticulum to the Golgi complex (By similarity). SUBUNIT: COPII is composed of at least five proteins: the Sec23/24 complex, the Sec13/31 complex and Sar1 (By similarity). SUBCELLULAR LOCATION: Golgi apparatus membrane (By similarity). Endoplasmic reticulum membrane (By similarity). Endoplasmic reticulum-Golgi intermediate compartment membrane (By similarity). DISEASE: Defects in SEC23B are the cause of congenital dyserythropoietic anemia type 2 (CDA2) [MIM:224100]. An autosomal recessive blood disorder characterized by morphological abnormalities of erythroblasts, ineffective erythropoiesis, normocytic anemia, iron overload, jaundice, and variable splenomegaly. Ultrastructural features include bi- or multinucleated erythroblasts in bone marrow, karyorrhexis, and the presence of Gaucher-like bone marrow histiocytes. The main biochemical feature of the disease is defective glycosylation of some red blood cells membrane proteins. SIMILARITY: Belongs to the SEC23/SEC24 family. SEC23 subfamily.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
SCOP Domains: 81811 - Helical domain of Sec23/24 81995 - beta-sandwich domain of Sec23/24 53300 - vWA-like 82754 - C-terminal, gelsolin-like domain of Sec23/24 82919 - Zn-finger domain of Sec23/24
ModBase Predicted Comparative 3D Structure on Q15437
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006886 intracellular protein transport GO:0006888 ER to Golgi vesicle-mediated transport GO:0015031 protein transport GO:0016192 vesicle-mediated transport GO:0090114 COPII-coated vesicle budding