Home > Product > Antibody > Rabbit Anti-SEC23B antibody
CDA II; CDAII; CDAN2; HEMPAS; Protein transport protein Sec23B; RP11-379J5.1; SC23B_HUMAN; Sec23 homolog B (S. cerevisiae); SEC23 related protein B; SEC23-like protein B; SEC23-related protein B; Sec23b; Transport protein SEC23B.
Cat:
SL19614R
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Pig,Horse,)
Immunogen:
KLH conjugated synthetic peptide derived from human SEC23B:501-600/767
Format:
Liquid
Storage instructions:
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500(Paraffin sections need to do antigen repair)not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
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Unit:
Price: $
Product PDFs
Datasheet:


The protein encoded by this gene is a member of the SEC23 subfamily of the SEC23/SEC24 family, which is involved in vesicle trafficking. The encoded protein has similarity to yeast Sec23p component of COPII. COPII is the coat protein complex responsible for vesicle budding from the ER. The function of this gene product has been implicated in cargo selection and concentration. Multiple alternatively spliced transcript variants have been identified in this gene. [provided by RefSeq, Feb 2010]

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.

Subcellular Location:
Golgi apparatus membrane. Endoplasmic reticulum membrane. Endoplasmic reticulum-Golgi intermediate compartment membrane.

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.

SWISS:
Q15437

Gene ID:
10483

Database links:

Entrez Gene: 10483 Human

Entrez Gene: 27054 Mouse

Entrez Gene: 362226 Rat

Omim: 610512 Human

SwissProt: Q15437 Human

SwissProt: Q9D662 Mouse

Unigene: 369373 Human

Unigene: 248492 Mouse



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