Target:CHST3
Fields:Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate
Gene Name:CHST3
Protein Name:Carbohydrate sulfotransferase 3 (EC 2.8.2.17) (Chondroitin 6-O-sulfotransferase 1) (C6ST-1) (Chondroitin 6-sulfotransferase) (Galactose/N-acetylglucosamine/N-acetylglucosamine 6-O-sulfotransferase 0)
Human Gene Id:9469
Human Swiss Prot No:Q7LGC8
Mouse Swiss Prot No:O88199
Rat Swiss Prot No:Q9QZL2
Immunogen:Synthesized peptide derived from part region of human protein
Specificity:CHST3 Polyclonal Antibody detects endogenous levels of protein.
Formulation:Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Source:Polyclonal, Rabbit,IgG
Dilution:WB 1:500-2000 ELISA 1:5000-20000
Purification:The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration:1 mg/ml
Storage Stability:-15°C to -25°C/1 year(Do not lower than -25°C)
Observed Band(KD):52kD
Background: This gene encodes an enzyme which catalyzes the sulfation of chondroitin, a proteoglycan found in the extracellular matrix and most cells which is involved in cell migration and differentiation. Mutations in this gene are associated with spondylepiphyseal dysplasia and humerospinal dysostosis. [provided by RefSeq, Mar 2009],
Function:catalytic activity:3'-phosphoadenylyl sulfate + chondroitin = adenosine 3',5'-bisphosphate + chondroitin 6'-sulfate.,disease:Defects in CHST3 are a cause of humerospinal dysostosis (HSD) [MIM:143095]. HSD is characterized by bifurcation of the ends of the humerus, subluxation in the elbow joints, widened iliac bones, talipes equinovarus and coronal cleft vertebrae. Congenital, progressive heart disease, possibly with fatal outcome, is observed in some patients.,disease:Defects in CHST3 are the cause of spondyloepiphyseal dysplasia Omani type (SED Omani type) [MIM:608637]. SED Omani type is an autosomal recessive disorder characterized by normal length at birth but severely reduced adult height (110-130 cm), severe progressive kyphoscoliosis, arthritic changes with joint dislocations, genu valgum, cubitus valgus, mild brachydactyly, camptodactyly, microdontia and normal intelligence. As a
Subcellular Location:Golgi apparatus membrane ; Single-pass type II membrane protein .
Expression:Widely expressed in adult tissues. Expressed in heart, placenta, skeletal muscle and pancreas. Also expressed in various immune tissues such as spleen, lymph node, thymus and appendix.