Cosmc, or C1GALT1-specific chaperone 1, is a 318 amino acid protein encoded by the human gene C1GALT1C1. Cosmc is believed to be a chaperone required for the generation of 1 O-glycan Gal-b1-3GalNAc-a1-Ser/Thr (Tn antigen), which is a precursor for many extended O-glycans in glycoproteins. Cosmc may also function as a specific molecular chaperone assisting the folding/stability of core 1 b-3-galactosyltransferase (C1GALT1). Cosmc is a single-pass type II membrane protein. Cosmc is ubiquitously expressed in all tissue types. It is most abundantly expressed in small intestine, stomach, salivary gland, kidney and testis, and at intermediate levels in whole brain, cerebellum, spinal cord, thymus, spleen, trachea, lung, pancreas, ovary and uterus. Defects in C1GALT1C1 are the cause of Tn syndrome, a rare autoimmune disease caused by somatic mutation in the C1GALT1C1 gene in which subpopulations of blood cells of all lineages carry an incompletely glycosylated Tn antigen, effecting red cells and platelets and leading to anemia, leukopenia and thrombocytopenia. Tn-polyagglutinability is sometimes associated with leukemia or is a preleukemic state.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFELISA
recommended for detection of Cosmc of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Alternative Names
epitope corresponding to amino acids 1-270 mapping at the N-terminus of Cosmc of human origin
Tissue Specificity
epitope corresponding to amino acids 1-270 mapping at the N-terminus of Cosmc of human origin
Western blot analysis of Cosmc expression in non-transfected: (A) and human Cosmc transfected: (B) 293T whole cell lysates.
Application Notes
recommended for detection of Cosmc of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine
Additional Information
Form
Liquid
Storage Instructions
For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.