Home > Product > Antibody > Rabbit Anti-SEC23B/FITC Conjugated 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-FITC
Species Reactivity:
(predicted: Human,Mouse,Rat,Dog,Pig,Horse,)
Immunogen:
KLH conjugated synthetic peptide derived from human SEC23B
Format:
Lyophilized or Liquid
Storage instructions:
Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant
Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Concentration:
1mg/ml
Clonality:
Polyclonal
Isotype:
IgG
Applications:
ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
Host:
Rabbit
Calculated MW:
86kDa
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Unit:
Price: $
Product PDFs
Datasheet:


background:
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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