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Product Name Anti-CHST14/AP Chinese Name 碱性磷酸酶(AP)标记的碳水化合物磺基转移酶14抗体 Alias 2600016L03Rik; ATCS; Carbohydrate (N acetylgalactosamine 4 0) sulfotransferase 14; Carbohydrate sulfotransferase 14; CHST14; CHSTE_HUMAN; D4ST-1; D4st1; Dermatan 4-sulfotransferase 1; hD4ST1; RP23-286G12.2; UNQ1925/PRO4400. Research Area Cell biology Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, Rat, (predicted: Human, Dog, Pig, Cow, Horse, Sheep, ) Applications WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 43kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human CHST14 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage 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 antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
This gene encodes a member of the HNK-1 family of sulfotransferases. The encoded protein transfers sulfate to the C-4 hydroxyl of N-acetylgalactosamine residues in dermatan sulfate. Mutations in this gene have been associated with adducted thumb-clubfoot syndrome.[provided by RefSeq, Mar 2010]
Function:
Catalyzes the transfer of sulfate to position 4 of the N-acetylgalactosamine (GalNAc) residue of dermatan sulfate. Transfers sulfate to the C-4 hydroxyl of beta1,4-linked GalNAc that is substituted with an alpha-linked iduronic acid (IdoUA) at the C-3 hydroxyl. Transfers sulfate more efficiently to GalNAc residues in -IdoUA-GalNAc-IdoUA- than in -GlcUA-GalNAc-GlcUA-sequences. Has preference for partially desulfated dermatan sulfate. Addition of sulfate to GalNAc may occur immediately after epimerization of GlcUA to IdoUA.
Subcellular Location:
Golgi apparatus membrane.
Tissue Specificity:
Widely expressed. Expressed at high level in pituitary gland, placenta, uterus and thyroid.
DISEASE:
Defects in CHST14 are the cause of Ehlers-Danlos syndrome musculocontractural type (EDSMC) [MIM:601776]. It is a form of Ehlers-Danlos syndrome characterized by distinctive craniofacial dysmorphism, congenital contractures of thumbs and fingers, clubfeet, severe kyphoscoliosis, muscular hypotonia, hyperextensible thin skin with easy bruisability and atrophic scarring, wrinkled palms, joint hypermobility, and ocular involvement.
Similarity:
Belongs to the sulfotransferase 2 family.
Database links:Entrez Gene: 113189 Human
Entrez Gene: 72136 Mouse
Omim: 608429 Human
SwissProt: Q8NCH0 Human
SwissProt: Q80V53 Mouse
Unigene: 442449 Human
Unigene: 278349 Mouse
Unigene: 482404 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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